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module path_id path_name name class description compounds
1 M00001_C00022 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
2 M00001_C00022 hsa00010 Glycolysis / Gluconeogenesis Glycolysis / Gluconeogenesis - Homo sapiens (human) Metabolism; Carbohydrate metabolism Glycolysis is the process of converting glucose into pyruvate and generating small amounts of ATP (energy) and NADH (reducing power). It is a central pathway that produces important precursor metabolites: six-carbon compounds of glucose-6P and fructose-6P and three-carbon compounds of glycerone-P, glyceraldehyde-3P, glycerate-3P, phosphoenolpyruvate, and pyruvate [MD:M00001]. Acetyl-CoA, another important precursor metabolite, is produced by oxidative decarboxylation of pyruvate [MD:M00307]. When the enzyme genes of this pathway are examined in completely sequenced genomes, the reaction steps of three-carbon compounds from glycerone-P to pyruvate form a conserved core module [MD:M00002], which is found in almost all organisms and which sometimes contains operon structures in bacterial genomes. Gluconeogenesis is a synthesis pathway of glucose from noncarbohydrate precursors. It is essentially a reversal of glycolysis with minor variations of alternative paths [MD:M00003]. Pyruvate, Acetyl-CoA, D-Glucose, Acetate, Oxaloacetate, Thiamin diphosphate, Phosphoenolpyruvate, Acetaldehyde, D-Fructose 6-phosphate, D-Glucose 1-phosphate, Glycerone phosphate, D-Glyceraldehyde 3-phosphate, (S)-Lactate, 3-Phospho-D-glycerate, beta-D-Glucose, 3-Phospho-D-glyceroyl phosphate, alpha-D-Glucose, D-Fructose 1,6-bisphosphate, Ethanol, 2-Phospho-D-glycerate, alpha-D-Glucose 6-phosphate, 2,3-Bisphospho-D-glycerate, beta-D-Glucose 6-phosphate, Salicin, 2-(alpha-Hydroxyethyl)thiamine diphosphate, Arbutin, Arbutin 6-phosphate, Salicin 6-phosphate, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine
3 M00002_C00022 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
4 M00002_C00022 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
5 M00002_C00022 hsa00010 Glycolysis / Gluconeogenesis Glycolysis / Gluconeogenesis - Homo sapiens (human) Metabolism; Carbohydrate metabolism Glycolysis is the process of converting glucose into pyruvate and generating small amounts of ATP (energy) and NADH (reducing power). It is a central pathway that produces important precursor metabolites: six-carbon compounds of glucose-6P and fructose-6P and three-carbon compounds of glycerone-P, glyceraldehyde-3P, glycerate-3P, phosphoenolpyruvate, and pyruvate [MD:M00001]. Acetyl-CoA, another important precursor metabolite, is produced by oxidative decarboxylation of pyruvate [MD:M00307]. When the enzyme genes of this pathway are examined in completely sequenced genomes, the reaction steps of three-carbon compounds from glycerone-P to pyruvate form a conserved core module [MD:M00002], which is found in almost all organisms and which sometimes contains operon structures in bacterial genomes. Gluconeogenesis is a synthesis pathway of glucose from noncarbohydrate precursors. It is essentially a reversal of glycolysis with minor variations of alternative paths [MD:M00003]. Pyruvate, Acetyl-CoA, D-Glucose, Acetate, Oxaloacetate, Thiamin diphosphate, Phosphoenolpyruvate, Acetaldehyde, D-Fructose 6-phosphate, D-Glucose 1-phosphate, Glycerone phosphate, D-Glyceraldehyde 3-phosphate, (S)-Lactate, 3-Phospho-D-glycerate, beta-D-Glucose, 3-Phospho-D-glyceroyl phosphate, alpha-D-Glucose, D-Fructose 1,6-bisphosphate, Ethanol, 2-Phospho-D-glycerate, alpha-D-Glucose 6-phosphate, 2,3-Bisphospho-D-glycerate, beta-D-Glucose 6-phosphate, Salicin, 2-(alpha-Hydroxyethyl)thiamine diphosphate, Arbutin, Arbutin 6-phosphate, Salicin 6-phosphate, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine
6 M00003_C05345 hsa00010 Glycolysis / Gluconeogenesis Glycolysis / Gluconeogenesis - Homo sapiens (human) Metabolism; Carbohydrate metabolism Glycolysis is the process of converting glucose into pyruvate and generating small amounts of ATP (energy) and NADH (reducing power). It is a central pathway that produces important precursor metabolites: six-carbon compounds of glucose-6P and fructose-6P and three-carbon compounds of glycerone-P, glyceraldehyde-3P, glycerate-3P, phosphoenolpyruvate, and pyruvate [MD:M00001]. Acetyl-CoA, another important precursor metabolite, is produced by oxidative decarboxylation of pyruvate [MD:M00307]. When the enzyme genes of this pathway are examined in completely sequenced genomes, the reaction steps of three-carbon compounds from glycerone-P to pyruvate form a conserved core module [MD:M00002], which is found in almost all organisms and which sometimes contains operon structures in bacterial genomes. Gluconeogenesis is a synthesis pathway of glucose from noncarbohydrate precursors. It is essentially a reversal of glycolysis with minor variations of alternative paths [MD:M00003]. Pyruvate, Acetyl-CoA, D-Glucose, Acetate, Oxaloacetate, Thiamin diphosphate, Phosphoenolpyruvate, Acetaldehyde, D-Fructose 6-phosphate, D-Glucose 1-phosphate, Glycerone phosphate, D-Glyceraldehyde 3-phosphate, (S)-Lactate, 3-Phospho-D-glycerate, beta-D-Glucose, 3-Phospho-D-glyceroyl phosphate, alpha-D-Glucose, D-Fructose 1,6-bisphosphate, Ethanol, 2-Phospho-D-glycerate, alpha-D-Glucose 6-phosphate, 2,3-Bisphospho-D-glycerate, beta-D-Glucose 6-phosphate, Salicin, 2-(alpha-Hydroxyethyl)thiamine diphosphate, Arbutin, Arbutin 6-phosphate, Salicin 6-phosphate, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine
7 M00003_C05345 hsa00020 Citrate cycle (TCA cycle) Citrate cycle (TCA cycle) - Homo sapiens (human) Metabolism; Carbohydrate metabolism The citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form of acetate, derived from glycolysis and pyruvate oxidation for carbohydrates and from beta oxidation of fatty acids. The two-carbon acetyl group in acetyl-CoA is transferred to the four-carbon compound of oxaloacetate to form the six-carbon compound of citrate. In a series of reactions two carbons in citrate are oxidized to CO2 and the reaction pathway supplies NADH for use in the oxidative phosphorylation and other metabolic processes. The pathway also supplies important precursor metabolites including 2-oxoglutarate. At the end of the cycle the remaining four-carbon part is transformed back to oxaloacetate. According to the genome sequence data, many organisms seem to lack genes for the full cycle [MD:M00009], but contain genes for specific segments [MD:M00010 M00011]. Pyruvate, Acetyl-CoA, 2-Oxoglutarate, Oxaloacetate, Succinate, Thiamin diphosphate, Phosphoenolpyruvate, Succinyl-CoA, Fumarate, (S)-Malate, Citrate, Isocitrate, cis-Aconitate, 2-(alpha-Hydroxyethyl)thiamine diphosphate, Oxalosuccinate, 3-Carboxy-1-hydroxypropyl-ThPP, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, [Dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine, [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine
8 M00004_R02739 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
9 M00004_R02739 hsa00030 Pentose phosphate pathway Pentose phosphate pathway - Homo sapiens (human) Metabolism; Carbohydrate metabolism The pentose phosphate pathway is a process of glucose turnover that produces NADPH as reducing equivalents and pentoses as essential parts of nucleotides. There are two different phases in the pathway. One is irreversible oxidative phase in which glucose-6P is converted to ribulose-5P by oxidative decarboxylation, and NADPH is generated [MD:M00006]. The other is reversible non-oxidative phase in which phosphorylated sugars are interconverted to generate xylulose-5P, ribulose-5P, and ribose-5P [MD:M00007]. Phosphoribosyl pyrophosphate (PRPP) formed from ribose-5P [MD:M00005] is an activated compound used in the biosynthesis of histidine and purine/pyrimidine nucleotides. This pathway map also shows the Entner-Doudoroff pathway where 6-P-gluconate is dehydrated and then cleaved into pyruvate and glyceraldehyde-3P [MD:M00008]. Pyruvate, D-Glucose, D-Fructose 6-phosphate, D-Ribose 5-phosphate, D-Glyceraldehyde 3-phosphate, 5-Phospho-alpha-D-ribose 1-diphosphate, D-Ribose, 3-Phospho-D-glycerate, D-Glucono-1,5-lactone, D-Ribulose 5-phosphate, 2-Dehydro-3-deoxy-D-gluconate, beta-D-Glucose, D-Xylulose 5-phosphate, D-Gluconic acid, D-Glycerate, D-Erythrose 4-phosphate, 6-Phospho-D-gluconate, D-Fructose 1,6-bisphosphate, D-Glyceraldehyde, alpha-D-Ribose 1-phosphate, 2-Phospho-D-glycerate, alpha-D-Glucose 6-phosphate, 2-Deoxy-D-ribose 1-phosphate, 2-Deoxy-D-ribose 5-phosphate, D-Ribose 1,5-bisphosphate, beta-D-Glucose 6-phosphate, D-Ribulose 1,5-bisphosphate, 6-Phospho-2-dehydro-D-gluconate, D-Glucono-1,5-lactone 6-phosphate, Deoxyribose, Sedoheptulose, 2-Amino-2-deoxy-D-gluconate, 2-Dehydro-3-deoxy-6-phospho-D-gluconate, Sedoheptulose 7-phosphate, D-arabino-Hex-3-ulose 6-phosphate, 2-Keto-D-gluconic acid, D-Glucosaminate-6-phosphate
10 M00006_C00199 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
11 M00006_C00199 hsa00030 Pentose phosphate pathway Pentose phosphate pathway - Homo sapiens (human) Metabolism; Carbohydrate metabolism The pentose phosphate pathway is a process of glucose turnover that produces NADPH as reducing equivalents and pentoses as essential parts of nucleotides. There are two different phases in the pathway. One is irreversible oxidative phase in which glucose-6P is converted to ribulose-5P by oxidative decarboxylation, and NADPH is generated [MD:M00006]. The other is reversible non-oxidative phase in which phosphorylated sugars are interconverted to generate xylulose-5P, ribulose-5P, and ribose-5P [MD:M00007]. Phosphoribosyl pyrophosphate (PRPP) formed from ribose-5P [MD:M00005] is an activated compound used in the biosynthesis of histidine and purine/pyrimidine nucleotides. This pathway map also shows the Entner-Doudoroff pathway where 6-P-gluconate is dehydrated and then cleaved into pyruvate and glyceraldehyde-3P [MD:M00008]. Pyruvate, D-Glucose, D-Fructose 6-phosphate, D-Ribose 5-phosphate, D-Glyceraldehyde 3-phosphate, 5-Phospho-alpha-D-ribose 1-diphosphate, D-Ribose, 3-Phospho-D-glycerate, D-Glucono-1,5-lactone, D-Ribulose 5-phosphate, 2-Dehydro-3-deoxy-D-gluconate, beta-D-Glucose, D-Xylulose 5-phosphate, D-Gluconic acid, D-Glycerate, D-Erythrose 4-phosphate, 6-Phospho-D-gluconate, D-Fructose 1,6-bisphosphate, D-Glyceraldehyde, alpha-D-Ribose 1-phosphate, 2-Phospho-D-glycerate, alpha-D-Glucose 6-phosphate, 2-Deoxy-D-ribose 1-phosphate, 2-Deoxy-D-ribose 5-phosphate, D-Ribose 1,5-bisphosphate, beta-D-Glucose 6-phosphate, D-Ribulose 1,5-bisphosphate, 6-Phospho-2-dehydro-D-gluconate, D-Glucono-1,5-lactone 6-phosphate, Deoxyribose, Sedoheptulose, 2-Amino-2-deoxy-D-gluconate, 2-Dehydro-3-deoxy-6-phospho-D-gluconate, Sedoheptulose 7-phosphate, D-arabino-Hex-3-ulose 6-phosphate, 2-Keto-D-gluconic acid, D-Glucosaminate-6-phosphate
12 M00007_C00117 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
13 M00007_C00117 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
14 M00007_C00117 hsa00030 Pentose phosphate pathway Pentose phosphate pathway - Homo sapiens (human) Metabolism; Carbohydrate metabolism The pentose phosphate pathway is a process of glucose turnover that produces NADPH as reducing equivalents and pentoses as essential parts of nucleotides. There are two different phases in the pathway. One is irreversible oxidative phase in which glucose-6P is converted to ribulose-5P by oxidative decarboxylation, and NADPH is generated [MD:M00006]. The other is reversible non-oxidative phase in which phosphorylated sugars are interconverted to generate xylulose-5P, ribulose-5P, and ribose-5P [MD:M00007]. Phosphoribosyl pyrophosphate (PRPP) formed from ribose-5P [MD:M00005] is an activated compound used in the biosynthesis of histidine and purine/pyrimidine nucleotides. This pathway map also shows the Entner-Doudoroff pathway where 6-P-gluconate is dehydrated and then cleaved into pyruvate and glyceraldehyde-3P [MD:M00008]. Pyruvate, D-Glucose, D-Fructose 6-phosphate, D-Ribose 5-phosphate, D-Glyceraldehyde 3-phosphate, 5-Phospho-alpha-D-ribose 1-diphosphate, D-Ribose, 3-Phospho-D-glycerate, D-Glucono-1,5-lactone, D-Ribulose 5-phosphate, 2-Dehydro-3-deoxy-D-gluconate, beta-D-Glucose, D-Xylulose 5-phosphate, D-Gluconic acid, D-Glycerate, D-Erythrose 4-phosphate, 6-Phospho-D-gluconate, D-Fructose 1,6-bisphosphate, D-Glyceraldehyde, alpha-D-Ribose 1-phosphate, 2-Phospho-D-glycerate, alpha-D-Glucose 6-phosphate, 2-Deoxy-D-ribose 1-phosphate, 2-Deoxy-D-ribose 5-phosphate, D-Ribose 1,5-bisphosphate, beta-D-Glucose 6-phosphate, D-Ribulose 1,5-bisphosphate, 6-Phospho-2-dehydro-D-gluconate, D-Glucono-1,5-lactone 6-phosphate, Deoxyribose, Sedoheptulose, 2-Amino-2-deoxy-D-gluconate, 2-Dehydro-3-deoxy-6-phospho-D-gluconate, Sedoheptulose 7-phosphate, D-arabino-Hex-3-ulose 6-phosphate, 2-Keto-D-gluconic acid, D-Glucosaminate-6-phosphate
15 M00009_R00342,R00361 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
16 M00009_R00342,R00361 hsa00020 Citrate cycle (TCA cycle) Citrate cycle (TCA cycle) - Homo sapiens (human) Metabolism; Carbohydrate metabolism The citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form of acetate, derived from glycolysis and pyruvate oxidation for carbohydrates and from beta oxidation of fatty acids. The two-carbon acetyl group in acetyl-CoA is transferred to the four-carbon compound of oxaloacetate to form the six-carbon compound of citrate. In a series of reactions two carbons in citrate are oxidized to CO2 and the reaction pathway supplies NADH for use in the oxidative phosphorylation and other metabolic processes. The pathway also supplies important precursor metabolites including 2-oxoglutarate. At the end of the cycle the remaining four-carbon part is transformed back to oxaloacetate. According to the genome sequence data, many organisms seem to lack genes for the full cycle [MD:M00009], but contain genes for specific segments [MD:M00010 M00011]. Pyruvate, Acetyl-CoA, 2-Oxoglutarate, Oxaloacetate, Succinate, Thiamin diphosphate, Phosphoenolpyruvate, Succinyl-CoA, Fumarate, (S)-Malate, Citrate, Isocitrate, cis-Aconitate, 2-(alpha-Hydroxyethyl)thiamine diphosphate, Oxalosuccinate, 3-Carboxy-1-hydroxypropyl-ThPP, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, [Dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine, [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine
17 M00010_C00026 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
18 M00010_C00026 hsa01210 2-Oxocarboxylic acid metabolism 2-Oxocarboxylic acid metabolism - Homo sapiens (human) NA 2-Oxocarboxylic acids, also called 2-oxo acids and alpha-keto acids, are the most elementary set of metabolites that includes pyruvate (2-oxopropanoate), 2-oxobutanoate, oxaloacetate (2-oxosuccinate) and 2-oxoglutarate. This diagram illustrates the architecture of chain extension and modification reaction modules for 2-oxocarboxylic acids. The chain extension module RM001 is a tricarboxylic pathway where acetyl-CoA derived carbon is used to extend the chain length by one. The chain modification modules RM002 (including RM032) and RM033, together with a reductive amination step (RC00006 or RC00036), generate basic and branched-chain amino acids, respectively. The modification module RM030 is used in the biosynthesis of glucosinolates, a class of plant secondary metabolites, for conversion to oxime followed by addition of thio-glucose moiety. Furthermore, the chain extension from 2-oxoadipate to 2-oxosuberate is followed by coenzyme B biosynthesis in methonogenic archaea. NA
19 M00010_C00026 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
20 M00010_C00026 hsa00020 Citrate cycle (TCA cycle) Citrate cycle (TCA cycle) - Homo sapiens (human) Metabolism; Carbohydrate metabolism The citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form of acetate, derived from glycolysis and pyruvate oxidation for carbohydrates and from beta oxidation of fatty acids. The two-carbon acetyl group in acetyl-CoA is transferred to the four-carbon compound of oxaloacetate to form the six-carbon compound of citrate. In a series of reactions two carbons in citrate are oxidized to CO2 and the reaction pathway supplies NADH for use in the oxidative phosphorylation and other metabolic processes. The pathway also supplies important precursor metabolites including 2-oxoglutarate. At the end of the cycle the remaining four-carbon part is transformed back to oxaloacetate. According to the genome sequence data, many organisms seem to lack genes for the full cycle [MD:M00009], but contain genes for specific segments [MD:M00010 M00011]. Pyruvate, Acetyl-CoA, 2-Oxoglutarate, Oxaloacetate, Succinate, Thiamin diphosphate, Phosphoenolpyruvate, Succinyl-CoA, Fumarate, (S)-Malate, Citrate, Isocitrate, cis-Aconitate, 2-(alpha-Hydroxyethyl)thiamine diphosphate, Oxalosuccinate, 3-Carboxy-1-hydroxypropyl-ThPP, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, [Dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine, [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine
21 M00011_C00036 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
22 M00011_C00036 hsa00020 Citrate cycle (TCA cycle) Citrate cycle (TCA cycle) - Homo sapiens (human) Metabolism; Carbohydrate metabolism The citrate cycle (TCA cycle, Krebs cycle) is an important aerobic pathway for the final steps of the oxidation of carbohydrates and fatty acids. The cycle starts with acetyl-CoA, the activated form of acetate, derived from glycolysis and pyruvate oxidation for carbohydrates and from beta oxidation of fatty acids. The two-carbon acetyl group in acetyl-CoA is transferred to the four-carbon compound of oxaloacetate to form the six-carbon compound of citrate. In a series of reactions two carbons in citrate are oxidized to CO2 and the reaction pathway supplies NADH for use in the oxidative phosphorylation and other metabolic processes. The pathway also supplies important precursor metabolites including 2-oxoglutarate. At the end of the cycle the remaining four-carbon part is transformed back to oxaloacetate. According to the genome sequence data, many organisms seem to lack genes for the full cycle [MD:M00009], but contain genes for specific segments [MD:M00010 M00011]. Pyruvate, Acetyl-CoA, 2-Oxoglutarate, Oxaloacetate, Succinate, Thiamin diphosphate, Phosphoenolpyruvate, Succinyl-CoA, Fumarate, (S)-Malate, Citrate, Isocitrate, cis-Aconitate, 2-(alpha-Hydroxyethyl)thiamine diphosphate, Oxalosuccinate, 3-Carboxy-1-hydroxypropyl-ThPP, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, [Dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine, [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine
23 M00013_C00024 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
24 M00013_C00024 hsa00640 Propanoate metabolism Propanoate metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA Acetyl-CoA, Oxaloacetate, Succinate, Thiamin diphosphate, Malonyl-CoA, Succinyl-CoA, beta-Alanine, Propanoyl-CoA, 2-Oxobutanoate, Glycerone phosphate, Propanoate, (S)-Lactate, 3-Oxopropanoate, (S)-Lactaldehyde, Propanal, Acrylic acid, Methylglyoxal, Propane-1,2-diol, (S)-Methylmalonyl-CoA, Propynoate, Lactoyl-CoA, Propenoyl-CoA, 3-Hydroxypropanoate, (R)-Methylmalonyl-CoA, Methylmalonate, 2-Methylcitrate, beta-Alanyl-CoA, Propanoyl phosphate, (Z)-But-2-ene-1,2,3-tricarboxylate, (2S,3R)-3-Hydroxybutane-1,2,3-tricarboxylate, Hydroxyacetone, 3-Hydroxypropionyl-CoA, Propane-1-ol, Propionyladenylate, 2-Hydroxybutanoic acid, 3-Oxopropionyl-CoA, (S)-Methylmalonate semialdehyde, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, 2-(alpha-Hydroxypropyl)thiamine diphosphate, Enzyme N6-(S-propyldihydrolipoyl)lysine, 2-Methyl-trans-aconitate
25 M00014_C00231 hsa00040 Pentose and glucuronate interconversions Pentose and glucuronate interconversions - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA Pyruvate, 2-Oxoglutarate, UDP-glucose, D-Glucose 1-phosphate, Glycerone phosphate, Glycerol, UDP-glucuronate, D-Xylose, D-Glucuronate, D-Ribulose 5-phosphate, 2-Dehydro-3-deoxy-D-gluconate, D-Arabinose, D-Xylulose 5-phosphate, L-Arabinose, Glycolaldehyde, D-Ribulose, D-Xylulose, L-Xylulose, D-Galacturonate, Xylitol, 2,5-Dioxopentanoate, Pectate, Ribitol, D-Lyxose, D-Xylonate, L-Ribulose, D-Mannonate, L-Arabitol, D-Tagaturonate, 3-Dehydro-L-gulonate, Pectin, CDP-ribitol, L-Gulonate, D-Altronate, D-Fructuronate, D-Ribitol 5-phosphate, L-Ribulose 5-phosphate, L-Lyxose, D-Arabitol, D-Xylonolactone, Digalacturonate, L-Glyceraldehyde, D-Xylono-1,4-lactone, beta-D-Glucuronoside, L-Xylulose 5-phosphate, 2-Dehydro-3-deoxy-D-xylonate, 5-Dehydro-4-deoxy-D-glucuronate, (4S)-4,6-Dihydroxy-2,5-dioxohexanoate, (4R,5S)-4,5,6-Trihydroxy-2,3-dioxohexanoate, D-Glucuronate 1-phosphate, L-Xylonate, L-Lyxonate, 4-(4-Deoxy-alpha-D-gluc-4-enuronosyl)-D-galacturonate, L-Xylulose 1-phosphate, 3-Dehydro-L-gulonate 6-phosphate, L-Galactonate, 2-Dehydro-3-deoxy-L-galactonate, D-Ribulose 1-phosphate, 4-Deoxy-L-threo-hex-4-enopyranuronate
26 M00015_C00148 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
27 M00015_C00148 hsa00330 Arginine and proline metabolism Arginine and proline metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Peptide, S-Adenosyl-L-methionine, Pyruvate, L-Glutamate, Glyoxylate, L-Arginine, L-Ornithine, Urea, Putrescine, L-Proline, Agmatine, Sarcosine, p-Coumaroyl-CoA, Creatine, Spermidine, 4-Aminobutanoate, Feruloyl-CoA, 5-Aminopentanoate, N-Carbamoylputrescine, L-Aspartate 4-semialdehyde, Nitric oxide, 4-Aminobutyraldehyde, Guanidinoacetate, Spermine, D-Proline, Creatinine, Homocarnosine, 1,3-Diaminopropane, 4-Guanidinobutanoate, N-Carbamoylsarcosine, 5-Amino-2-oxopentanoic acid, S-Adenosylmethioninamine, Hydroxyproline, L-Glutamate 5-semialdehyde, N-Acetyl-L-glutamate 5-semialdehyde, Nopaline, Phosphocreatine, N-Methylhydantoin, 4-Guanidinobutanal, N-Acetylputrescine, 4-Acetamidobutanoate, 4-Guanidinobutanamide, Phosphoguanidinoacetate, L-Glutamyl 5-phosphate, N2-Succinyl-L-arginine, Norspermidine, N2-Succinyl-L-ornithine, 1-Pyrroline-2-carboxylate, 5-Guanidino-2-oxopentanoate, (S)-1-Pyrroline-5-carboxylate, Octopine, L-1-Pyrroline-3-hydroxy-5-carboxylate, trans-3-Hydroxy-L-proline, N-Succinyl-L-glutamate, N-Succinyl-L-glutamate 5-semialdehyde, N(omega)-Hydroxyarginine, N4-Acetylaminobutanal, L-4-Hydroxyglutamate semialdehyde, L-Arginine phosphate, (4R)-4-Hydroxy-2-oxoglutarate, L-erythro-4-Hydroxyglutamate, Feruloylputrescine, gamma-L-Glutamylputrescine, gamma-Glutamyl-gamma-aminobutyraldehyde, 4-(L-gamma-Glutamylamino)butanoate, Carboxyspermidine, Carboxynorspermidine, p-Coumaroylputrescine, cis-3-Hydroxy-L-proline, N1-(3-Aminopropyl)agmatine, N1-[(S)-3-amino-3-carboxypropyl]agmatine
28 M00020_C00065 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
29 M00020_C00065 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
30 M00020_C00065 hsa00260 Glycine, serine and threonine metabolism Glycine, serine and threonine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism Serine is derived from 3-phospho-D-glycerate, an intermediate of glycolysis [MD:M00020], and glycine is derived from serine. Threonine is an essential amino acid, which animals cannot synthesize. In bacteria and plants, threonine is derived from aspartate [MD:M00018]. CO2, Ammonia, Pyruvate, Glycine, Glyoxylate, L-Aspartate, L-Serine, L-Tryptophan, L-Cysteine, Tetrahydrofolate, 2-Oxobutanoate, Choline, 5,10-Methylenetetrahydrofolate, Hydroxypyruvate, L-Threonine, 3-Phospho-D-glycerate, Sarcosine, D-Glycerate, L-Homoserine, Creatine, 5-Aminolevulinate, L-Aspartate 4-semialdehyde, Methylglyoxal, Betaine aldehyde, Guanidinoacetate, 2-Phospho-D-glycerate, Betaine, D-Serine, 1,3-Diaminopropane, O-Phospho-L-serine, N,N-Dimethylglycine, O-Phospho-L-homoserine, [Protein]-S8-aminomethyldihydrolipoyllysine, Aminoacetone, Lipoylprotein, L-Cystathionine, Phosphatidylserine, Dihydrolipoylprotein, 4-Phospho-L-aspartate, (R)-1-Aminopropan-2-ol, 3-Phosphonooxypyruvate, L-2,4-Diaminobutanoate, L-2-Amino-3-oxobutanoic acid, L-Allothreonine, Ectoine, N(gamma)-Acetyldiaminobutyrate, 5-Hydroxyectoine, N(alpha)-Acetyl-L-2,4-diaminobutyrate
31 M00027_C00042 hsa00250 Alanine, aspartate and glutamate metabolism Alanine, aspartate and glutamate metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Ammonia, Pyruvate, L-Glutamate, 2-Oxoglutarate, Oxaloacetate, L-Alanine, Succinate, L-Aspartate, L-Glutamine, Fumarate, L-Asparagine, Citrate, Carbamoyl phosphate, Succinate semialdehyde, 4-Aminobutanoate, D-Glucosamine 6-phosphate, D-Aspartate, N-Carbamoyl-L-aspartate, 2-Oxoglutaramate, N-Acetyl-L-aspartate, 2-Oxosuccinamate, 5-Phosphoribosylamine, N-(L-Arginino)succinate, N6-(1,2-Dicarboxyethyl)-AMP, (S)-1-Pyrroline-5-carboxylate, N-Acetylaspartylglutamate, beta-Citryl-L-glutamate, N-Acetylaspartylglutamylglutamate
32 M00029_C00122 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
33 M00029_C00122 hsa00220 Arginine biosynthesis Arginine biosynthesis - Homo sapiens (human) Metabolism; Amino acid metabolism NA CO2, Ammonia, L-Glutamate, 2-Oxoglutarate, L-Aspartate, L-Arginine, L-Glutamine, L-Ornithine, Urea, Fumarate, Carbamoyl phosphate, L-Citrulline, N-Acetylornithine, N-Acetyl-L-glutamate, Urea-1-carboxylate, N-Acetyl-L-glutamate 5-semialdehyde, N-(L-Arginino)succinate, N-Acetyl-L-glutamate 5-phosphate, N-Acetyl-L-citrulline, LysW-L-glutamate, LysW-L-glutamyl 5-phosphate, LysW-L-glutamate 5-semialdehyde, LysW-L-ornithine
34 M00029_C00086 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
35 M00029_C00086 hsa00220 Arginine biosynthesis Arginine biosynthesis - Homo sapiens (human) Metabolism; Amino acid metabolism NA CO2, Ammonia, L-Glutamate, 2-Oxoglutarate, L-Aspartate, L-Arginine, L-Glutamine, L-Ornithine, Urea, Fumarate, Carbamoyl phosphate, L-Citrulline, N-Acetylornithine, N-Acetyl-L-glutamate, Urea-1-carboxylate, N-Acetyl-L-glutamate 5-semialdehyde, N-(L-Arginino)succinate, N-Acetyl-L-glutamate 5-phosphate, N-Acetyl-L-citrulline, LysW-L-glutamate, LysW-L-glutamyl 5-phosphate, LysW-L-glutamate 5-semialdehyde, LysW-L-ornithine
36 M00032_C00332 hsa00310 Lysine degradation Lysine degradation - Homo sapiens (human) Metabolism; Amino acid metabolism NA Acetyl-CoA, 2-Oxoglutarate, Glycine, Succinate, L-Lysine, Thiamin diphosphate, Butanoyl-CoA, Acetoacetate, 2-Oxoadipate, Acetoacetyl-CoA, L-Pipecolate, 5-Aminopentanoate, N6-(L-1,3-Dicarboxypropyl)-L-lysine, (S)-2,3,4,5-Tetrahydropyridine-2-carboxylate, Carnitine, Glutarate, Glutaryl-CoA, D-Lysine, Crotonoyl-CoA, L-2-Aminoadipate, 5-Aminopentanamide, (R)-2-Hydroxyglutarate, (3S)-3,6-Diaminohexanoate, (S)-3-Hydroxybutanoyl-CoA, 4-Trimethylammoniobutanal, 4-Trimethylammoniobutanoate, (3S,5S)-3,5-Diaminohexanoate, Procollagen 5-hydroxy-L-lysine, (3S)-3-Hydroxy-N6,N6,N6-trimethyl-L-lysine, Cadaverine, Protein lysine, N6-Acetyl-L-lysine, 5-Acetamidopentanoate, (S)-2-Hydroxyglutarate, 6-Amino-2-oxohexanoate, 5-Oxopentanoate, 5-Phosphooxy-L-lysine, (S)-5-Amino-3-oxohexanoic acid, N6,N6,N6-Trimethyl-L-lysine, D-Lysopine, L-2-Aminoadipate 6-semialdehyde, Delta1-Piperideine-2-carboxylate, 5-(D-Galactosyloxy)-L-lysine-procollagen, (2R,5S)-2,5-Diaminohexanoate, L-3-Aminobutyryl-CoA, Protein N6-methyl-L-lysine, Protein N6,N6-dimethyl-L-lysine, Protein N6,N6,N6-trimethyl-L-lysine, 6-Acetamido-2-oxohexanoate, 2-Amino-5-oxohexanoate, [Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysine, 5-Aminopentanal, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, L-Hydroxylysine, 4-Carboxy-1-hydroxybutyryl-ThPP
37 M00034_C00147 hsa00270 Cysteine and methionine metabolism Cysteine and methionine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism Cysteine and methionine are sulfur-containing amino acids. Cysteine is synthesized from serine through different pathways in different organism groups. In bacteria and plants, cysteine is converted from serine (via acetylserine) by transfer of hydrogen sulfide [MD:M00021]. In animals, methionine-derived homocysteine is used as sulfur source and its condensation product with serine (cystathionine) is converted to cysteine [MD:M00338]. Cysteine is metabolized to pyruvate in multiple routes. Methionine is an essential amino acid, which animals cannot synthesize. In bacteria and plants, methionine is synthesized from aspartate [MD:M00017]. S-Adenosylmethionine (SAM), synthesized from methionine and ATP, is a methyl group donor in many important transfer reactions including DNA methylation for regulation of gene expression. SAM may also be used to regenerate methionine in the methionine salvage pathway [MD:M00034]. S-Adenosyl-L-methionine, S-Adenosyl-L-homocysteine, Pyruvate, L-Alanine, L-Aspartate, Glutathione, Sulfate, L-Serine, L-Methionine, Sulfite, L-Cysteine, 2-Oxobutanoate, L-Homocysteine, 5'-Methylthioadenosine, 3-Phospho-D-glycerate, L-Homoserine, Hydrogen sulfide, Methanethiol, L-Aspartate 4-semialdehyde, L-Cystine, L-Cysteate, 3-Sulfino-L-alanine, D-Cysteine, Mercaptopyruvate, O-Acetyl-L-serine, O-Phospho-L-serine, O-Acetyl-L-homoserine, O-Phospho-L-homoserine, O-Succinyl-L-homoserine, S-Adenosylmethioninamine, 4-Methylthio-2-oxobutanoic acid, 1-Aminocyclopropane-1-carboxylate, L-Homocystine, Thiocysteine, Dehydroalanine, L-Cystathionine, (S)-2-Aminobutanoate, L-Methionine S-oxide, 4-Phospho-L-aspartate, 5-Methylthio-D-ribose, N-Formylmethionine, 3-Phosphonooxypyruvate, S-Inosyl-L-homocysteine, S-Ribosyl-L-homocysteine, S-Methyl-5-thio-D-ribose 1-phosphate, S-Methyl-5-thio-D-ribulose 1-phosphate, Nicotianamine, Aminoacyl-L-methionine, S-Glutathionyl-L-cysteine, 3-Sulfinylpyruvate, 3-Sulfopyruvate, 3-Mercaptolactate, S-Sulfo-L-cysteine, Ethylene, 3-(Methylthio)propanoate, Sulfur dioxide, 1-Deoxy-D-xylulose 5-phosphate, HSO3-, (S)-3-Sulfolactate, (2R)-3-Sulfolactate, 1,2-Dihydroxy-5-(methylthio)pent-1-en-3-one, 2,3-Diketo-5-methylthiopentyl-1-phosphate, 2-Hydroxy-3-keto-5-methylthiopentenyl-1-phosphate, N-Acyl-L-homoserine lactone, 5'-S-Methyl-5'-thioinosine, gamma-L-Glutamyl-L-2-aminobutyrate, Ophthalmate, S-Methyl-1-thio-D-xylulose 5-phosphate
38 M00035_C02291 hsa00270 Cysteine and methionine metabolism Cysteine and methionine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism Cysteine and methionine are sulfur-containing amino acids. Cysteine is synthesized from serine through different pathways in different organism groups. In bacteria and plants, cysteine is converted from serine (via acetylserine) by transfer of hydrogen sulfide [MD:M00021]. In animals, methionine-derived homocysteine is used as sulfur source and its condensation product with serine (cystathionine) is converted to cysteine [MD:M00338]. Cysteine is metabolized to pyruvate in multiple routes. Methionine is an essential amino acid, which animals cannot synthesize. In bacteria and plants, methionine is synthesized from aspartate [MD:M00017]. S-Adenosylmethionine (SAM), synthesized from methionine and ATP, is a methyl group donor in many important transfer reactions including DNA methylation for regulation of gene expression. SAM may also be used to regenerate methionine in the methionine salvage pathway [MD:M00034]. S-Adenosyl-L-methionine, S-Adenosyl-L-homocysteine, Pyruvate, L-Alanine, L-Aspartate, Glutathione, Sulfate, L-Serine, L-Methionine, Sulfite, L-Cysteine, 2-Oxobutanoate, L-Homocysteine, 5'-Methylthioadenosine, 3-Phospho-D-glycerate, L-Homoserine, Hydrogen sulfide, Methanethiol, L-Aspartate 4-semialdehyde, L-Cystine, L-Cysteate, 3-Sulfino-L-alanine, D-Cysteine, Mercaptopyruvate, O-Acetyl-L-serine, O-Phospho-L-serine, O-Acetyl-L-homoserine, O-Phospho-L-homoserine, O-Succinyl-L-homoserine, S-Adenosylmethioninamine, 4-Methylthio-2-oxobutanoic acid, 1-Aminocyclopropane-1-carboxylate, L-Homocystine, Thiocysteine, Dehydroalanine, L-Cystathionine, (S)-2-Aminobutanoate, L-Methionine S-oxide, 4-Phospho-L-aspartate, 5-Methylthio-D-ribose, N-Formylmethionine, 3-Phosphonooxypyruvate, S-Inosyl-L-homocysteine, S-Ribosyl-L-homocysteine, S-Methyl-5-thio-D-ribose 1-phosphate, S-Methyl-5-thio-D-ribulose 1-phosphate, Nicotianamine, Aminoacyl-L-methionine, S-Glutathionyl-L-cysteine, 3-Sulfinylpyruvate, 3-Sulfopyruvate, 3-Mercaptolactate, S-Sulfo-L-cysteine, Ethylene, 3-(Methylthio)propanoate, Sulfur dioxide, 1-Deoxy-D-xylulose 5-phosphate, HSO3-, (S)-3-Sulfolactate, (2R)-3-Sulfolactate, 1,2-Dihydroxy-5-(methylthio)pent-1-en-3-one, 2,3-Diketo-5-methylthiopentyl-1-phosphate, 2-Hydroxy-3-keto-5-methylthiopentenyl-1-phosphate, N-Acyl-L-homoserine lactone, 5'-S-Methyl-5'-thioinosine, gamma-L-Glutamyl-L-2-aminobutyrate, Ophthalmate, S-Methyl-1-thio-D-xylulose 5-phosphate
39 M00036_C00164 hsa00280 Valine, leucine and isoleucine degradation Valine, leucine and isoleucine degradation - Homo sapiens (human) Metabolism; Amino acid metabolism NA Acetyl-CoA, Thiamin diphosphate, Succinyl-CoA, Propanoyl-CoA, L-Leucine, 3-Methyl-2-oxobutanoic acid, Acetoacetate, L-Valine, 4-Methyl-2-oxopentanoate, Acetoacetyl-CoA, 2-Methyl-3-oxopropanoate, (S)-3-Hydroxy-3-methylglutaryl-CoA, L-Isoleucine, 2-Methylpropanoyl-CoA, (S)-3-Methyl-2-oxopentanoic acid, (S)-Methylmalonyl-CoA, (R)-3-Amino-2-methylpropanoate, (R)-Methylmalonyl-CoA, Methylmalonate, 3-Methylbutanoyl-CoA, 3-Methylcrotonyl-CoA, 3-Methylglutaconyl-CoA, L-3-Aminoisobutanoate, 2-Methylacetoacetyl-CoA, 2-Methylbut-2-enoyl-CoA, 2-Methylprop-2-enoyl-CoA, (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA, Branched chain fatty acid, 3-Hydroxyisovaleryl-CoA, (S)-3-Hydroxyisobutyryl-CoA, (S)-3-Hydroxyisobutyrate, (S)-Methylmalonate semialdehyde, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, 3-Methyl-1-hydroxybutyl-ThPP, [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(3-methylbutanoyl)dihydrolipoyllysine, 2-Methyl-1-hydroxypropyl-ThPP, [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine, 2-Methyl-1-hydroxybutyl-ThPP, [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylbutanoyl)dihydrolipoyllysine, (S)-2-Methylbutanoyl-CoA, 3-Hydroxyisovalerate
40 M00037_C01598 hsa00380 Tryptophan metabolism Tryptophan metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Acetyl-CoA, L-Tryptophan, Anthranilate, 2-Oxoadipate, L-Kynurenine, Indolepyruvate, Acetoacetyl-CoA, Tryptamine, Indole, Glutaryl-CoA, 3-Hydroxyanthranilate, Indole-3-acetaldehyde, 5-Hydroxy-L-tryptophan, Serotonin, Crotonoyl-CoA, Indole-3-acetate, Indole-3-ethanol, N-Acetylserotonin, 7,8-Dihydroxykynurenate, (S)-3-Hydroxybutanoyl-CoA, 7,8-Dihydro-7,8-dihydroxykynurenate, 4-(2-Aminophenyl)-2,4-dioxobutanoate, Melatonin, 4-Hydroxy-2-quinolinecarboxylic acid, 2-Aminophenol, 2-Aminophenoxazin-3-one, N-Acetylisatin, 2-Aminomuconate, N-Acetylindoxyl, Xanthurenic acid, (Indol-3-yl)acetamide, L-Formylkynurenine, 2,3-Dihydroxyindole, Indole-3-acetaldehyde oxime, 3-Indoleacetonitrile, 3-Hydroxy-L-kynurenine, (Indol-3-yl)glycolaldehyde, gamma-Oxalocrotonate, 2-Formylaminobenzaldehyde, Quinolinate, 2-Aminomuconate semialdehyde, 2-Amino-3-carboxymuconate semialdehyde, 5-Hydroxyindoleacetaldehyde, 5-Hydroxyindoleacetate, 3-Hydroxykynurenamine, 4,8-Dihydroxyquinoline, 5-Hydroxykynurenamine, 4,6-Dihydroxyquinoline, Cinnavalininate, 5-(3'-Carboxy-3'-oxopropenyl)-4,6-dihydroxypicolinate, Formyl-N-acetyl-5-methoxykynurenamine, 6-Hydroxymelatonin, 3-Methylindolepyruvate, 4-(2-Amino-3-hydroxyphenyl)-2,4-dioxobutanoate, 5-Hydroxyindolepyruvate, Formyl-5-hydroxykynurenamine, 5-Hydroxy-N-formylkynurenine, 5-Hydroxykynurenine, 4-(2-Amino-5-hydroxyphenyl)-2,4-dioxobutanoate, Formylanthranilate, 5-(2'-Formylethyl)-4,6-dihydroxypicolinate, 5-(2'-Carboxyethyl)-4,6-dihydroxypicolinate, 5-(3'-Carboxy-3'-oxopropyl)-4,6-dihydroxypicolinate, Indoxyl, 5-Methoxytryptamine, 5-Methoxyindoleacetate, 6-Hydroxykynurenate, 8-Methoxykynurenate, 3-Methoxyanthranilate, 5-Hydroxyindoleacetylglycine, 3-Methyldioxyindole, 2-Formaminobenzoylacetate, Glucobrassicin, N-Methylserotonin, N-Methyltryptamine, Skatole, Picolinic acid, Indolylmethylthiohydroximate, Indolylmethyl-desulfoglucosinolate, S-(Indolylmethylthiohydroximoyl)-L-cysteine, N-Hydroxyl-tryptamine, (R)-(Indol-3-yl)lactate, 2-Oxoindole-3-acetate
41 M00042_C00788 hsa00350 Tyrosine metabolism Tyrosine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Pyruvate, Succinate, L-Tyrosine, Fumarate, Phenol, Acetoacetate, Succinate semialdehyde, 3,4-Dihydroxy-L-phenylalanine, Tyramine, Hydroquinone, Homogentisate, L-Noradrenaline, 2,5-Dihydroxybenzoate, 4-Hydroxyphenylacetate, L-Adrenaline, 4-Coumarate, Dopaquinone, 4-Maleylacetoacetate, 3,5-Diiodo-L-tyrosine, 4-Fumarylacetoacetate, 3,4-Dihydroxyphenylacetate, 3-(4-Hydroxyphenyl)pyruvate, Maleic acid, L-Dopachrome, Thyroxine, Rosmarinate, Maleylpyruvate, N-Methyltyramine, Triiodothyronine, 3-Fumarylpyruvate, 3-Iodo-L-tyrosine, 2-Oxohept-3-enedioate, 4-Chlorophenylacetate, Dopamine, 4-Hydroxyphenylacetaldehyde, (R)-3-(4-Hydroxyphenyl)lactate, 3,4-Dihydroxyphenylacetaldehyde, 3-(3,4-Dihydroxyphenyl)pyruvate, 5-Carboxy-2-oxohept-3-enedioate, 5,6-Dihydroxyindole-2-carboxylate, 5-Carboxymethyl-2-hydroxymuconate, 3-Amino-3-(4-hydroxyphenyl)propanoate, 2-Hydroxy-5-carboxymethylmuconate semialdehyde, 4-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 5-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 4-Hydroxyphenylacetyl-CoA, 2-Hydroxy-3-(4-hydroxyphenyl)propenoate, 3,4-Dihydroxyphenylethyleneglycol, 3,4-Dihydroxymandelaldehyde, 5,6-Dihydroxyindole, Indole-5,6-quinone, 3,4-Dihydroxymandelate, 3-Methoxy-4-hydroxyphenylacetaldehyde, Homovanillate, 3-Methoxy-4-hydroxyphenylglycolaldehyde, 3-Methoxy-4-hydroxymandelate, Gentisate aldehyde, 3-Methoxytyramine, L-Metanephrine, L-Normetanephrine, 3-Hydroxyphenylacetate, 3-Methoxy-4-hydroxyphenylethyleneglycol, 4-Hydroxyphenylacetylglutamic acid, p-Hydroxyphenylacetylglycine, 2-Hydroxyhepta-2,4-dienedioate, 2-Carboxy-2,3-dihydro-5,6-dihydroxyindole, 4-Hydroxyphenylethanol, Salidroside, Stizolobate, Stizolobinate, Hordenine, 2,4-Dihydroxyhept-2-enedioate, 3,4-Dihydroxyphenylpropanoate, Cysteinyldopa, Phaeomelanin, Eumelanin, 5,6-Indolequinone-2-carboxylic acid, (R)-3-(3,4-Dihydroxyphenyl)lactate
42 M00043_C02465 hsa00350 Tyrosine metabolism Tyrosine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Pyruvate, Succinate, L-Tyrosine, Fumarate, Phenol, Acetoacetate, Succinate semialdehyde, 3,4-Dihydroxy-L-phenylalanine, Tyramine, Hydroquinone, Homogentisate, L-Noradrenaline, 2,5-Dihydroxybenzoate, 4-Hydroxyphenylacetate, L-Adrenaline, 4-Coumarate, Dopaquinone, 4-Maleylacetoacetate, 3,5-Diiodo-L-tyrosine, 4-Fumarylacetoacetate, 3,4-Dihydroxyphenylacetate, 3-(4-Hydroxyphenyl)pyruvate, Maleic acid, L-Dopachrome, Thyroxine, Rosmarinate, Maleylpyruvate, N-Methyltyramine, Triiodothyronine, 3-Fumarylpyruvate, 3-Iodo-L-tyrosine, 2-Oxohept-3-enedioate, 4-Chlorophenylacetate, Dopamine, 4-Hydroxyphenylacetaldehyde, (R)-3-(4-Hydroxyphenyl)lactate, 3,4-Dihydroxyphenylacetaldehyde, 3-(3,4-Dihydroxyphenyl)pyruvate, 5-Carboxy-2-oxohept-3-enedioate, 5,6-Dihydroxyindole-2-carboxylate, 5-Carboxymethyl-2-hydroxymuconate, 3-Amino-3-(4-hydroxyphenyl)propanoate, 2-Hydroxy-5-carboxymethylmuconate semialdehyde, 4-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 5-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 4-Hydroxyphenylacetyl-CoA, 2-Hydroxy-3-(4-hydroxyphenyl)propenoate, 3,4-Dihydroxyphenylethyleneglycol, 3,4-Dihydroxymandelaldehyde, 5,6-Dihydroxyindole, Indole-5,6-quinone, 3,4-Dihydroxymandelate, 3-Methoxy-4-hydroxyphenylacetaldehyde, Homovanillate, 3-Methoxy-4-hydroxyphenylglycolaldehyde, 3-Methoxy-4-hydroxymandelate, Gentisate aldehyde, 3-Methoxytyramine, L-Metanephrine, L-Normetanephrine, 3-Hydroxyphenylacetate, 3-Methoxy-4-hydroxyphenylethyleneglycol, 4-Hydroxyphenylacetylglutamic acid, p-Hydroxyphenylacetylglycine, 2-Hydroxyhepta-2,4-dienedioate, 2-Carboxy-2,3-dihydro-5,6-dihydroxyindole, 4-Hydroxyphenylethanol, Salidroside, Stizolobate, Stizolobinate, Hordenine, 2,4-Dihydroxyhept-2-enedioate, 3,4-Dihydroxyphenylpropanoate, Cysteinyldopa, Phaeomelanin, Eumelanin, 5,6-Indolequinone-2-carboxylic acid, (R)-3-(3,4-Dihydroxyphenyl)lactate
43 M00044_C00164 hsa00350 Tyrosine metabolism Tyrosine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Pyruvate, Succinate, L-Tyrosine, Fumarate, Phenol, Acetoacetate, Succinate semialdehyde, 3,4-Dihydroxy-L-phenylalanine, Tyramine, Hydroquinone, Homogentisate, L-Noradrenaline, 2,5-Dihydroxybenzoate, 4-Hydroxyphenylacetate, L-Adrenaline, 4-Coumarate, Dopaquinone, 4-Maleylacetoacetate, 3,5-Diiodo-L-tyrosine, 4-Fumarylacetoacetate, 3,4-Dihydroxyphenylacetate, 3-(4-Hydroxyphenyl)pyruvate, Maleic acid, L-Dopachrome, Thyroxine, Rosmarinate, Maleylpyruvate, N-Methyltyramine, Triiodothyronine, 3-Fumarylpyruvate, 3-Iodo-L-tyrosine, 2-Oxohept-3-enedioate, 4-Chlorophenylacetate, Dopamine, 4-Hydroxyphenylacetaldehyde, (R)-3-(4-Hydroxyphenyl)lactate, 3,4-Dihydroxyphenylacetaldehyde, 3-(3,4-Dihydroxyphenyl)pyruvate, 5-Carboxy-2-oxohept-3-enedioate, 5,6-Dihydroxyindole-2-carboxylate, 5-Carboxymethyl-2-hydroxymuconate, 3-Amino-3-(4-hydroxyphenyl)propanoate, 2-Hydroxy-5-carboxymethylmuconate semialdehyde, 4-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 5-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 4-Hydroxyphenylacetyl-CoA, 2-Hydroxy-3-(4-hydroxyphenyl)propenoate, 3,4-Dihydroxyphenylethyleneglycol, 3,4-Dihydroxymandelaldehyde, 5,6-Dihydroxyindole, Indole-5,6-quinone, 3,4-Dihydroxymandelate, 3-Methoxy-4-hydroxyphenylacetaldehyde, Homovanillate, 3-Methoxy-4-hydroxyphenylglycolaldehyde, 3-Methoxy-4-hydroxymandelate, Gentisate aldehyde, 3-Methoxytyramine, L-Metanephrine, L-Normetanephrine, 3-Hydroxyphenylacetate, 3-Methoxy-4-hydroxyphenylethyleneglycol, 4-Hydroxyphenylacetylglutamic acid, p-Hydroxyphenylacetylglycine, 2-Hydroxyhepta-2,4-dienedioate, 2-Carboxy-2,3-dihydro-5,6-dihydroxyindole, 4-Hydroxyphenylethanol, Salidroside, Stizolobate, Stizolobinate, Hordenine, 2,4-Dihydroxyhept-2-enedioate, 3,4-Dihydroxyphenylpropanoate, Cysteinyldopa, Phaeomelanin, Eumelanin, 5,6-Indolequinone-2-carboxylic acid, (R)-3-(3,4-Dihydroxyphenyl)lactate
44 M00044_C00122 hsa00350 Tyrosine metabolism Tyrosine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Pyruvate, Succinate, L-Tyrosine, Fumarate, Phenol, Acetoacetate, Succinate semialdehyde, 3,4-Dihydroxy-L-phenylalanine, Tyramine, Hydroquinone, Homogentisate, L-Noradrenaline, 2,5-Dihydroxybenzoate, 4-Hydroxyphenylacetate, L-Adrenaline, 4-Coumarate, Dopaquinone, 4-Maleylacetoacetate, 3,5-Diiodo-L-tyrosine, 4-Fumarylacetoacetate, 3,4-Dihydroxyphenylacetate, 3-(4-Hydroxyphenyl)pyruvate, Maleic acid, L-Dopachrome, Thyroxine, Rosmarinate, Maleylpyruvate, N-Methyltyramine, Triiodothyronine, 3-Fumarylpyruvate, 3-Iodo-L-tyrosine, 2-Oxohept-3-enedioate, 4-Chlorophenylacetate, Dopamine, 4-Hydroxyphenylacetaldehyde, (R)-3-(4-Hydroxyphenyl)lactate, 3,4-Dihydroxyphenylacetaldehyde, 3-(3,4-Dihydroxyphenyl)pyruvate, 5-Carboxy-2-oxohept-3-enedioate, 5,6-Dihydroxyindole-2-carboxylate, 5-Carboxymethyl-2-hydroxymuconate, 3-Amino-3-(4-hydroxyphenyl)propanoate, 2-Hydroxy-5-carboxymethylmuconate semialdehyde, 4-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 5-(L-Alanin-3-yl)-2-hydroxy-cis,cis-muconate 6-semialdehyde, 4-Hydroxyphenylacetyl-CoA, 2-Hydroxy-3-(4-hydroxyphenyl)propenoate, 3,4-Dihydroxyphenylethyleneglycol, 3,4-Dihydroxymandelaldehyde, 5,6-Dihydroxyindole, Indole-5,6-quinone, 3,4-Dihydroxymandelate, 3-Methoxy-4-hydroxyphenylacetaldehyde, Homovanillate, 3-Methoxy-4-hydroxyphenylglycolaldehyde, 3-Methoxy-4-hydroxymandelate, Gentisate aldehyde, 3-Methoxytyramine, L-Metanephrine, L-Normetanephrine, 3-Hydroxyphenylacetate, 3-Methoxy-4-hydroxyphenylethyleneglycol, 4-Hydroxyphenylacetylglutamic acid, p-Hydroxyphenylacetylglycine, 2-Hydroxyhepta-2,4-dienedioate, 2-Carboxy-2,3-dihydro-5,6-dihydroxyindole, 4-Hydroxyphenylethanol, Salidroside, Stizolobate, Stizolobinate, Hordenine, 2,4-Dihydroxyhept-2-enedioate, 3,4-Dihydroxyphenylpropanoate, Cysteinyldopa, Phaeomelanin, Eumelanin, 5,6-Indolequinone-2-carboxylic acid, (R)-3-(3,4-Dihydroxyphenyl)lactate
45 M00046_C00099 hsa00240 Pyrimidine metabolism Pyrimidine metabolism - Homo sapiens (human) Metabolism; Nucleotide metabolism NA UDP, DNA, RNA, CMP, CTP, L-Glutamine, UTP, Urea, beta-Alanine, UMP, Uracil, CDP, 5-Phospho-alpha-D-ribose 1-diphosphate, Carbamoyl phosphate, Thymine, Thymidine, 3-Oxopropanoate, dCMP, Orotate, Uridine, (S)-Dihydroorotate, dTDP, dTMP, dUMP, Cytosine, Malonate, 5,6-Dihydrouracil, N-Carbamoyl-L-aspartate, dCTP, dTTP, dUTP, Cytidine, Deoxyuridine, 2-Deoxy-D-ribose 1-phosphate, dCDP, Barbiturate, Deoxycytidine, 3',5'-Cyclic CMP, 3-Hydroxypropanoate, Orotidine 5'-phosphate, Pseudouridine 5'-phosphate, (R)-3-Amino-2-methylpropanoate, dUDP, 3'-UMP, Carbamate, Pseudouridine, Methylmalonate, 2',3'-Cyclic CMP, 2',3'-Cyclic UMP, 5-Methylcytosine, 3-Ureidopropionate, 5-Hydroxymethyldeoxycytidylate, 5-Methylbarbiturate, 3'-CMP, P1,P4-Bis(5'-uridyl) tetraphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-diphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-triphosphate, 3-Oxo-3-ureidopropanoate, (Z)-3-Ureidoacrylate peracid, (Z)-3-Peroxyaminoacrylate, Aminoacrylate, Ureidoacrylate, 3-Oxo-3-ureidoisobutyrate, (R)-5,6-Dihydrothymine, (R)-3-Ureidoisobutyrate, 3',5'-Cyclic UMP
46 M00046_C01205 hsa00240 Pyrimidine metabolism Pyrimidine metabolism - Homo sapiens (human) Metabolism; Nucleotide metabolism NA UDP, DNA, RNA, CMP, CTP, L-Glutamine, UTP, Urea, beta-Alanine, UMP, Uracil, CDP, 5-Phospho-alpha-D-ribose 1-diphosphate, Carbamoyl phosphate, Thymine, Thymidine, 3-Oxopropanoate, dCMP, Orotate, Uridine, (S)-Dihydroorotate, dTDP, dTMP, dUMP, Cytosine, Malonate, 5,6-Dihydrouracil, N-Carbamoyl-L-aspartate, dCTP, dTTP, dUTP, Cytidine, Deoxyuridine, 2-Deoxy-D-ribose 1-phosphate, dCDP, Barbiturate, Deoxycytidine, 3',5'-Cyclic CMP, 3-Hydroxypropanoate, Orotidine 5'-phosphate, Pseudouridine 5'-phosphate, (R)-3-Amino-2-methylpropanoate, dUDP, 3'-UMP, Carbamate, Pseudouridine, Methylmalonate, 2',3'-Cyclic CMP, 2',3'-Cyclic UMP, 5-Methylcytosine, 3-Ureidopropionate, 5-Hydroxymethyldeoxycytidylate, 5-Methylbarbiturate, 3'-CMP, P1,P4-Bis(5'-uridyl) tetraphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-diphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-triphosphate, 3-Oxo-3-ureidopropanoate, (Z)-3-Ureidoacrylate peracid, (Z)-3-Peroxyaminoacrylate, Aminoacrylate, Ureidoacrylate, 3-Oxo-3-ureidoisobutyrate, (R)-5,6-Dihydrothymine, (R)-3-Ureidoisobutyrate, 3',5'-Cyclic UMP
47 M00047_C00791 hsa00330 Arginine and proline metabolism Arginine and proline metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Peptide, S-Adenosyl-L-methionine, Pyruvate, L-Glutamate, Glyoxylate, L-Arginine, L-Ornithine, Urea, Putrescine, L-Proline, Agmatine, Sarcosine, p-Coumaroyl-CoA, Creatine, Spermidine, 4-Aminobutanoate, Feruloyl-CoA, 5-Aminopentanoate, N-Carbamoylputrescine, L-Aspartate 4-semialdehyde, Nitric oxide, 4-Aminobutyraldehyde, Guanidinoacetate, Spermine, D-Proline, Creatinine, Homocarnosine, 1,3-Diaminopropane, 4-Guanidinobutanoate, N-Carbamoylsarcosine, 5-Amino-2-oxopentanoic acid, S-Adenosylmethioninamine, Hydroxyproline, L-Glutamate 5-semialdehyde, N-Acetyl-L-glutamate 5-semialdehyde, Nopaline, Phosphocreatine, N-Methylhydantoin, 4-Guanidinobutanal, N-Acetylputrescine, 4-Acetamidobutanoate, 4-Guanidinobutanamide, Phosphoguanidinoacetate, L-Glutamyl 5-phosphate, N2-Succinyl-L-arginine, Norspermidine, N2-Succinyl-L-ornithine, 1-Pyrroline-2-carboxylate, 5-Guanidino-2-oxopentanoate, (S)-1-Pyrroline-5-carboxylate, Octopine, L-1-Pyrroline-3-hydroxy-5-carboxylate, trans-3-Hydroxy-L-proline, N-Succinyl-L-glutamate, N-Succinyl-L-glutamate 5-semialdehyde, N(omega)-Hydroxyarginine, N4-Acetylaminobutanal, L-4-Hydroxyglutamate semialdehyde, L-Arginine phosphate, (4R)-4-Hydroxy-2-oxoglutarate, L-erythro-4-Hydroxyglutamate, Feruloylputrescine, gamma-L-Glutamylputrescine, gamma-Glutamyl-gamma-aminobutyraldehyde, 4-(L-gamma-Glutamylamino)butanoate, Carboxyspermidine, Carboxynorspermidine, p-Coumaroylputrescine, cis-3-Hydroxy-L-proline, N1-(3-Aminopropyl)agmatine, N1-[(S)-3-amino-3-carboxypropyl]agmatine
48 M00048_C00130 hsa00230 Purine metabolism Purine metabolism - Homo sapiens (human) Metabolism; Nucleotide metabolism NA ATP, ADP, CO2, Ammonia, AMP, GDP, Glycine, DNA, GTP, RNA, Glyoxylate, 3'-Phosphoadenylyl sulfate, Adenosine 3',5'-bisphosphate, Sulfate, L-Glutamine, ITP, Urea, IDP, D-Ribose 5-phosphate, 5-Phospho-alpha-D-ribose 1-diphosphate, IMP, dATP, GMP, Adenine, Carbamoyl phosphate, dADP, Oxalate, Adenosine, Adenylyl sulfate, Guanine, Hypoxanthine, dGTP, HCO3-, Inosine, ADP-ribose, Deoxyguanosine, dAMP, dGDP, dGMP, Urate, Xanthine, Guanosine, Allantoate, Deoxyadenosine, 3',5'-Cyclic AMP, (S)-Ureidoglycolate, alpha-D-Ribose 1-phosphate, Xanthosine 5'-phosphate, XTP, Oxalureate, 3',5'-Cyclic GMP, Guanosine 3',5'-bis(diphosphate), P1,P4-Bis(5'-adenosyl)tetraphosphate, P1,P4-Bis(5'-guanosyl) tetraphosphate, dIDP, dITP, 3'-AMP, Oxamate, Xanthosine, (S)-Ureidoglycine, (R)(-)-Allantoin, (S)-Allantoin, 2',3'-Cyclic AMP, N-Formiminoglycine, 5-Phosphoribosylamine, Aminoimidazole ribotide, Adenosine tetraphosphate, Inosine 5'-tetraphosphate, N6-(1,2-Dicarboxyethyl)-AMP, 5'-Phosphoribosylglycinamide, 5-Amino-4-imidazolecarboxyamide, 5'-Phosphoribosyl-N-formylglycinamide, P1,P4-Bis(5'-xanthosyl) tetraphosphate, Guanosine 3'-diphosphate 5'-triphosphate, 2-(Formamido)-N1-(5'-phosphoribosyl)acetamidine, 1-(5'-Phosphoribosyl)-5-amino-4-imidazolecarboxamide, 1-(5'-Phosphoribosyl)-5-formamido-4-imidazolecarboxamide, 1-(5-Phospho-D-ribosyl)-5-amino-4-imidazolecarboxylate, 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole, 5-Aminoimidazole, Deoxyinosine, Urate-3-ribonucleoside, 5-Ureido-4-imidazole carboxylate, 5-Amino-4-imidazole carboxylate, Acetyl adenylate, Guanosine 3'-phosphate, 2',3'-Cyclic GMP, Imidazolone, 2'-Deoxyinosine 5'-phosphate, P1,P3-Bis(5'-adenosyl) triphosphate, 5'-Benzoylphosphoadenosine, 5'-Butyrylphosphoinosine, 5-Hydroxyisourate, 5-Hydroxy-2-oxo-4-ureido-2,5-dihydro-1H-imidazole-5-carboxylate, 5-Carboxyamino-1-(5-phospho-D-ribosyl)imidazole, N6-Succino-2-amino-2'-deoxyadenylate, dZMP, dZDP, dZTP, 8-Hydroxyadenine, 2,8-Dihydroxyadenine
49 M00049_C00002 hsa00230 Purine metabolism Purine metabolism - Homo sapiens (human) Metabolism; Nucleotide metabolism NA ATP, ADP, CO2, Ammonia, AMP, GDP, Glycine, DNA, GTP, RNA, Glyoxylate, 3'-Phosphoadenylyl sulfate, Adenosine 3',5'-bisphosphate, Sulfate, L-Glutamine, ITP, Urea, IDP, D-Ribose 5-phosphate, 5-Phospho-alpha-D-ribose 1-diphosphate, IMP, dATP, GMP, Adenine, Carbamoyl phosphate, dADP, Oxalate, Adenosine, Adenylyl sulfate, Guanine, Hypoxanthine, dGTP, HCO3-, Inosine, ADP-ribose, Deoxyguanosine, dAMP, dGDP, dGMP, Urate, Xanthine, Guanosine, Allantoate, Deoxyadenosine, 3',5'-Cyclic AMP, (S)-Ureidoglycolate, alpha-D-Ribose 1-phosphate, Xanthosine 5'-phosphate, XTP, Oxalureate, 3',5'-Cyclic GMP, Guanosine 3',5'-bis(diphosphate), P1,P4-Bis(5'-adenosyl)tetraphosphate, P1,P4-Bis(5'-guanosyl) tetraphosphate, dIDP, dITP, 3'-AMP, Oxamate, Xanthosine, (S)-Ureidoglycine, (R)(-)-Allantoin, (S)-Allantoin, 2',3'-Cyclic AMP, N-Formiminoglycine, 5-Phosphoribosylamine, Aminoimidazole ribotide, Adenosine tetraphosphate, Inosine 5'-tetraphosphate, N6-(1,2-Dicarboxyethyl)-AMP, 5'-Phosphoribosylglycinamide, 5-Amino-4-imidazolecarboxyamide, 5'-Phosphoribosyl-N-formylglycinamide, P1,P4-Bis(5'-xanthosyl) tetraphosphate, Guanosine 3'-diphosphate 5'-triphosphate, 2-(Formamido)-N1-(5'-phosphoribosyl)acetamidine, 1-(5'-Phosphoribosyl)-5-amino-4-imidazolecarboxamide, 1-(5'-Phosphoribosyl)-5-formamido-4-imidazolecarboxamide, 1-(5-Phospho-D-ribosyl)-5-amino-4-imidazolecarboxylate, 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole, 5-Aminoimidazole, Deoxyinosine, Urate-3-ribonucleoside, 5-Ureido-4-imidazole carboxylate, 5-Amino-4-imidazole carboxylate, Acetyl adenylate, Guanosine 3'-phosphate, 2',3'-Cyclic GMP, Imidazolone, 2'-Deoxyinosine 5'-phosphate, P1,P3-Bis(5'-adenosyl) triphosphate, 5'-Benzoylphosphoadenosine, 5'-Butyrylphosphoinosine, 5-Hydroxyisourate, 5-Hydroxy-2-oxo-4-ureido-2,5-dihydro-1H-imidazole-5-carboxylate, 5-Carboxyamino-1-(5-phospho-D-ribosyl)imidazole, N6-Succino-2-amino-2'-deoxyadenylate, dZMP, dZDP, dZTP, 8-Hydroxyadenine, 2,8-Dihydroxyadenine
50 M00050_C00044 hsa00230 Purine metabolism Purine metabolism - Homo sapiens (human) Metabolism; Nucleotide metabolism NA ATP, ADP, CO2, Ammonia, AMP, GDP, Glycine, DNA, GTP, RNA, Glyoxylate, 3'-Phosphoadenylyl sulfate, Adenosine 3',5'-bisphosphate, Sulfate, L-Glutamine, ITP, Urea, IDP, D-Ribose 5-phosphate, 5-Phospho-alpha-D-ribose 1-diphosphate, IMP, dATP, GMP, Adenine, Carbamoyl phosphate, dADP, Oxalate, Adenosine, Adenylyl sulfate, Guanine, Hypoxanthine, dGTP, HCO3-, Inosine, ADP-ribose, Deoxyguanosine, dAMP, dGDP, dGMP, Urate, Xanthine, Guanosine, Allantoate, Deoxyadenosine, 3',5'-Cyclic AMP, (S)-Ureidoglycolate, alpha-D-Ribose 1-phosphate, Xanthosine 5'-phosphate, XTP, Oxalureate, 3',5'-Cyclic GMP, Guanosine 3',5'-bis(diphosphate), P1,P4-Bis(5'-adenosyl)tetraphosphate, P1,P4-Bis(5'-guanosyl) tetraphosphate, dIDP, dITP, 3'-AMP, Oxamate, Xanthosine, (S)-Ureidoglycine, (R)(-)-Allantoin, (S)-Allantoin, 2',3'-Cyclic AMP, N-Formiminoglycine, 5-Phosphoribosylamine, Aminoimidazole ribotide, Adenosine tetraphosphate, Inosine 5'-tetraphosphate, N6-(1,2-Dicarboxyethyl)-AMP, 5'-Phosphoribosylglycinamide, 5-Amino-4-imidazolecarboxyamide, 5'-Phosphoribosyl-N-formylglycinamide, P1,P4-Bis(5'-xanthosyl) tetraphosphate, Guanosine 3'-diphosphate 5'-triphosphate, 2-(Formamido)-N1-(5'-phosphoribosyl)acetamidine, 1-(5'-Phosphoribosyl)-5-amino-4-imidazolecarboxamide, 1-(5'-Phosphoribosyl)-5-formamido-4-imidazolecarboxamide, 1-(5-Phospho-D-ribosyl)-5-amino-4-imidazolecarboxylate, 1-(5'-Phosphoribosyl)-5-amino-4-(N-succinocarboxamide)-imidazole, 5-Aminoimidazole, Deoxyinosine, Urate-3-ribonucleoside, 5-Ureido-4-imidazole carboxylate, 5-Amino-4-imidazole carboxylate, Acetyl adenylate, Guanosine 3'-phosphate, 2',3'-Cyclic GMP, Imidazolone, 2'-Deoxyinosine 5'-phosphate, P1,P3-Bis(5'-adenosyl) triphosphate, 5'-Benzoylphosphoadenosine, 5'-Butyrylphosphoinosine, 5-Hydroxyisourate, 5-Hydroxy-2-oxo-4-ureido-2,5-dihydro-1H-imidazole-5-carboxylate, 5-Carboxyamino-1-(5-phospho-D-ribosyl)imidazole, N6-Succino-2-amino-2'-deoxyadenylate, dZMP, dZDP, dZTP, 8-Hydroxyadenine, 2,8-Dihydroxyadenine
51 M00051_C00105 hsa00240 Pyrimidine metabolism Pyrimidine metabolism - Homo sapiens (human) Metabolism; Nucleotide metabolism NA UDP, DNA, RNA, CMP, CTP, L-Glutamine, UTP, Urea, beta-Alanine, UMP, Uracil, CDP, 5-Phospho-alpha-D-ribose 1-diphosphate, Carbamoyl phosphate, Thymine, Thymidine, 3-Oxopropanoate, dCMP, Orotate, Uridine, (S)-Dihydroorotate, dTDP, dTMP, dUMP, Cytosine, Malonate, 5,6-Dihydrouracil, N-Carbamoyl-L-aspartate, dCTP, dTTP, dUTP, Cytidine, Deoxyuridine, 2-Deoxy-D-ribose 1-phosphate, dCDP, Barbiturate, Deoxycytidine, 3',5'-Cyclic CMP, 3-Hydroxypropanoate, Orotidine 5'-phosphate, Pseudouridine 5'-phosphate, (R)-3-Amino-2-methylpropanoate, dUDP, 3'-UMP, Carbamate, Pseudouridine, Methylmalonate, 2',3'-Cyclic CMP, 2',3'-Cyclic UMP, 5-Methylcytosine, 3-Ureidopropionate, 5-Hydroxymethyldeoxycytidylate, 5-Methylbarbiturate, 3'-CMP, P1,P4-Bis(5'-uridyl) tetraphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-diphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-triphosphate, 3-Oxo-3-ureidopropanoate, (Z)-3-Ureidoacrylate peracid, (Z)-3-Peroxyaminoacrylate, Aminoacrylate, Ureidoacrylate, 3-Oxo-3-ureidoisobutyrate, (R)-5,6-Dihydrothymine, (R)-3-Ureidoisobutyrate, 3',5'-Cyclic UMP
52 M00052_C00112 hsa00240 Pyrimidine metabolism Pyrimidine metabolism - Homo sapiens (human) Metabolism; Nucleotide metabolism NA UDP, DNA, RNA, CMP, CTP, L-Glutamine, UTP, Urea, beta-Alanine, UMP, Uracil, CDP, 5-Phospho-alpha-D-ribose 1-diphosphate, Carbamoyl phosphate, Thymine, Thymidine, 3-Oxopropanoate, dCMP, Orotate, Uridine, (S)-Dihydroorotate, dTDP, dTMP, dUMP, Cytosine, Malonate, 5,6-Dihydrouracil, N-Carbamoyl-L-aspartate, dCTP, dTTP, dUTP, Cytidine, Deoxyuridine, 2-Deoxy-D-ribose 1-phosphate, dCDP, Barbiturate, Deoxycytidine, 3',5'-Cyclic CMP, 3-Hydroxypropanoate, Orotidine 5'-phosphate, Pseudouridine 5'-phosphate, (R)-3-Amino-2-methylpropanoate, dUDP, 3'-UMP, Carbamate, Pseudouridine, Methylmalonate, 2',3'-Cyclic CMP, 2',3'-Cyclic UMP, 5-Methylcytosine, 3-Ureidopropionate, 5-Hydroxymethyldeoxycytidylate, 5-Methylbarbiturate, 3'-CMP, P1,P4-Bis(5'-uridyl) tetraphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-diphosphate, 2'-Deoxy-5-hydroxymethylcytidine-5'-triphosphate, 3-Oxo-3-ureidopropanoate, (Z)-3-Ureidoacrylate peracid, (Z)-3-Peroxyaminoacrylate, Aminoacrylate, Ureidoacrylate, 3-Oxo-3-ureidoisobutyrate, (R)-5,6-Dihydrothymine, (R)-3-Ureidoisobutyrate, 3',5'-Cyclic UMP
53 M00055_G00008 hsa00510 N-Glycan biosynthesis N-Glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism N-glycans or asparagine-linked glycans are major constituents of glycoproteins in eukaryotes. N-glycans are covalently attached to asparagine with the consensus sequence of Asn-X-Ser/Thr by an N-glycosidic bond, GlcNAc b1- Asn. Biosynthesis of N-glycans begins on the cytoplasmic face of the ER membrane with the transferase reaction of UDP-GlcNAc and the lipid-like precursor P-Dol (dolichol phosphate) to generate GlcNAc a1- PP-Dol. After sequential addition of monosaccharides by ALG glycosyltransferases [MD:M00055], the N-glycan precursor is attached by the OST (oligosaccharyltransferase) complex to the polypeptide chain that is being synthesized and translocated through the ER membrane. The protein-bound N-glycan precursor is subsequently trimmed, extended, and modified in the ER and Golgi by a complex series of reactions catalyzed by membrane-bound glycosidases and glycosyltransferases. N-glycans thus synthesized are classified into three types: high-mannose type, complex type, and hybrid type. Defects in N-glycan biosynthesis lead to a variety of human diseases known as congenital disorders of glycosylation [DS:H00118 H00119]. GDP-mannose, Dolichyl phosphate, Dolichol, Dolichyl diphosphate, Dolichyl beta-D-glucosyl phosphate, Protein asparagine, Dolichyl phosphate D-mannose, Polyprenol, N-Acetyl-D-glucosaminyldiphosphodolichol, N,N'-Chitobiosyldiphosphodolichol, NA G00003, NA G00004, NA G00005, NA G00006, NA G00007, NA G00008, NA G00009, NA G00010, NA G00011, NA G00012, NA G00013, NA G00014, NA G00015, Core fucose, NA G00017, DS 3, NA G00019, NA G00020, NA G00021, NA G00022, NA G00171, NA G10526, NA G10595, NA G10596, NA G10597, NA G10598, NA G10599, NA G10694
54 M00056_G00025 hsa00512 O-Glycan biosynthesis Mucin type O-glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism O-glycans are a class of glycans that modify serine or threonine residues of proteins. Biosynthesis of O-glycans starts from the transfer of N-acetylgalactosamine (GalNAc) to serine or threonine. The first GalNAc may be extended with sugars including galactose, N-acetylglucosamine, fucose, or sialic acid, but not mannose, glucose, or xylose. Depending on the sugars added, there are four common O-glycan core structures, cores 1 through 4, and an additional four, cores 5 though 8. Mucins are highly O-glycosylated glycoproteins ubiquitous in mucous secretions on cell surfaces and in body fluids. Mucin O-glycans can be branched, and many sugars or groups of sugars are antigenic. Important modifications of mucin O-glycans include O-acetylation of sialic acid and O-sulfation of galactose and N-acetylglucosamine. [Protein]-L-serine, Tn antigen, Thomsen-Friedenreich antigen, NA G00025, Sialyl T antigen, NA G00027, NA G00028, NA G00029, NA G00030, NA G00031, NA G00032, NA G00033, NA G00034, Sialyl-Tn antigen, UDP-N-acetyl-D-galactosamine, NA G10794, NA G13153
55 M00056_G00029 hsa00512 O-Glycan biosynthesis Mucin type O-glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism O-glycans are a class of glycans that modify serine or threonine residues of proteins. Biosynthesis of O-glycans starts from the transfer of N-acetylgalactosamine (GalNAc) to serine or threonine. The first GalNAc may be extended with sugars including galactose, N-acetylglucosamine, fucose, or sialic acid, but not mannose, glucose, or xylose. Depending on the sugars added, there are four common O-glycan core structures, cores 1 through 4, and an additional four, cores 5 though 8. Mucins are highly O-glycosylated glycoproteins ubiquitous in mucous secretions on cell surfaces and in body fluids. Mucin O-glycans can be branched, and many sugars or groups of sugars are antigenic. Important modifications of mucin O-glycans include O-acetylation of sialic acid and O-sulfation of galactose and N-acetylglucosamine. [Protein]-L-serine, Tn antigen, Thomsen-Friedenreich antigen, NA G00025, Sialyl T antigen, NA G00027, NA G00028, NA G00029, NA G00030, NA G00031, NA G00032, NA G00033, NA G00034, Sialyl-Tn antigen, UDP-N-acetyl-D-galactosamine, NA G10794, NA G13153
56 M00056_G00031 hsa00512 O-Glycan biosynthesis Mucin type O-glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism O-glycans are a class of glycans that modify serine or threonine residues of proteins. Biosynthesis of O-glycans starts from the transfer of N-acetylgalactosamine (GalNAc) to serine or threonine. The first GalNAc may be extended with sugars including galactose, N-acetylglucosamine, fucose, or sialic acid, but not mannose, glucose, or xylose. Depending on the sugars added, there are four common O-glycan core structures, cores 1 through 4, and an additional four, cores 5 though 8. Mucins are highly O-glycosylated glycoproteins ubiquitous in mucous secretions on cell surfaces and in body fluids. Mucin O-glycans can be branched, and many sugars or groups of sugars are antigenic. Important modifications of mucin O-glycans include O-acetylation of sialic acid and O-sulfation of galactose and N-acetylglucosamine. [Protein]-L-serine, Tn antigen, Thomsen-Friedenreich antigen, NA G00025, Sialyl T antigen, NA G00027, NA G00028, NA G00029, NA G00030, NA G00031, NA G00032, NA G00033, NA G00034, Sialyl-Tn antigen, UDP-N-acetyl-D-galactosamine, NA G10794, NA G13153
57 M00057_G00157 hsa00532 Glycosaminoglycan biosynthesis - chondroitin sulfate Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism Glycosaminoglycans (GAGs) are linear polysaccharide chains consisting of repeating disaccharide units and form proteglycans by covalently attaching to their core proteins. Chondroitin sulfate (CS) is a glycosaminoglycan with the disaccharide unit of beta-D-galactosamine (GalNAc) and beta-D-glucuronic acid (GlcA), and often modified with ester-linked sulfate at certain positions. Dermatan sulfate (DS) is a modified form of CS, in which a portion of D-glucuronate residues is epimerized to L-iduronates (IdoA). CS and DS are linked to serine residues in core proteins via a linkage tetrasaccharide formed by the transfer of xylose and three more residues [MD:M00057]. The assembly process of CS is initiated by transferring GalNAc residue to the linkage tetrasaccharide. The polymerization is catalyzed by bifunctional enzymes (chondroitin synthases) possessing both beta 1,3 glucuronosyltransferase and beta 1,4 N-acetylgalactosaminyltransferase activities [MD:M00058]. Chondroitin polymerization also requires the action of the chondroitin polymerizing factor. There are various O-sulfation patterns in CS and DS, where 4-O sulfation and 6-O sulfation of GalNAc and 2-O-sulfation of the uronic acids (GlcA / IdoA) are mainly found. 3'-Phosphoadenylyl sulfate, Adenosine 3',5'-bisphosphate, Chondroitin, Dermatan, [Protein]-L-serine, NA G00154, NA G00155, NA G00156, NA G00157, NA G00158, NA G00159, NA G00160
58 M00058_G00160 hsa00532 Glycosaminoglycan biosynthesis - chondroitin sulfate Glycosaminoglycan biosynthesis - chondroitin sulfate / dermatan sulfate - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism Glycosaminoglycans (GAGs) are linear polysaccharide chains consisting of repeating disaccharide units and form proteglycans by covalently attaching to their core proteins. Chondroitin sulfate (CS) is a glycosaminoglycan with the disaccharide unit of beta-D-galactosamine (GalNAc) and beta-D-glucuronic acid (GlcA), and often modified with ester-linked sulfate at certain positions. Dermatan sulfate (DS) is a modified form of CS, in which a portion of D-glucuronate residues is epimerized to L-iduronates (IdoA). CS and DS are linked to serine residues in core proteins via a linkage tetrasaccharide formed by the transfer of xylose and three more residues [MD:M00057]. The assembly process of CS is initiated by transferring GalNAc residue to the linkage tetrasaccharide. The polymerization is catalyzed by bifunctional enzymes (chondroitin synthases) possessing both beta 1,3 glucuronosyltransferase and beta 1,4 N-acetylgalactosaminyltransferase activities [MD:M00058]. Chondroitin polymerization also requires the action of the chondroitin polymerizing factor. There are various O-sulfation patterns in CS and DS, where 4-O sulfation and 6-O sulfation of GalNAc and 2-O-sulfation of the uronic acids (GlcA / IdoA) are mainly found. 3'-Phosphoadenylyl sulfate, Adenosine 3',5'-bisphosphate, Chondroitin, Dermatan, [Protein]-L-serine, NA G00154, NA G00155, NA G00156, NA G00157, NA G00158, NA G00159, NA G00160
59 M00059_G00164 hsa00534 Glycosaminoglycan biosynthesis - heparan sulfate Glycosaminoglycan biosynthesis - heparan sulfate / heparin - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism Heparan sulfate (HS) and heparin (Hep) are glycosaminoglycans with repeating disaccharide units that consist of alternating residues of alpha-D-glucosamine (GlcN) and uronic acid, the latter being either beta-D-glucuronic acid (GlcA) or alpha-L-iduronic acid (IdoA). In these sugar residues, sulfation modification may be performed at various positions. Structural studies show that Hep possesses a higher degree of sulfation than HS. The biosynthesis of HS/Hep occurs with the addition of the first GlcNAc residue by EXTL3 glycosyltransferase after completion of tetrasaccharide linkage region attached to serine residue of a core protein. The chain polymeraization is then catalyzed by EXT1 and EXT2 transferases. As the chain polymerizes, HS/Hep undergoes a series of modification reactions including N-deacetylation, N-sulfation, epimerization, and subsequently O-sulfation. As final products of biosynthesis, HS is present in the form of hepran sulfate proteoglycan (HSPG) whereas Hep exists as a sugar chain without a core protein. The proteoglycan families with HS, as well as CS (chondroitin sulfate), DS (dermatan sulfate), and KS (keratan sulfate), are composed of two main types depending on the subcellular locations: cell membrane and extracellular matrix [BR:00535]. HS/Hep has been shown to bind to a variety of molecules, such as growth factors, chemokines, morphogens, and extracellular matrix components [BR:00536]. Acetate, 3'-Phosphoadenylyl sulfate, Adenosine 3',5'-bisphosphate, [Protein]-L-serine, NA G00154, NA G00155, NA G00156, NA G00157, NA G00162, NA G00163, NA G00164
60 M00065_G13044 hsa00563 Glycosylphosphatidylinositol(GPI)-anchor biosynthesis Glycosylphosphatidylinositol (GPI)-anchor biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism Cell surface proteins can be attached to the cell membrane via the glycolipid structure called glycosylphosphatidylinositol (GPI) anchor. Hundreds of GPI-anchored proteins have been identified in many eukaryotes ranging from protozoa and fungi to mammals. All protein-linked GPI anchors share a common core structure, characterized by the substructure Man (a1-4) GlcN (a1-6) myo-inositol-1P-lipid. Biosynthesis of GPI anchors proceeds in three stages: (i) preassembly of a GPI precursor in the ER membrane, (ii) attachment of the GPI to the C-terminus of a newly synthesized protein in the lumen of the ER, and (iii) lipid remodeling and/or carbohydrate side-chain modifications in the ER and the Golgi. Defects of GPI anchor biosynthesis gene result in a genetic disorder, paroxysmal nocturnal hemoglobinuria. Palmitoyl-CoA, Ethanolamine phosphate, Phosphatidylethanolamine, 1-Phosphatidyl-D-myo-inositol, NA G00143, NA G00144, NA G00145, NA G00146, NA G00148, NA G00149, UDP-N-acetyl-D-glucosamine, Dolichyl phosphate D-mannose, 6-(alpha-D-glucosaminyl)-1D-myo-inositol, NA G13044, NA G13046, NA G13128, NA G13150, NA G13151, NA G13152
61 M00066_C01290 hsa00600 Sphingolipid metabolism Sphingolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA L-Serine, Palmitoyl-CoA, N-Acylsphingosine, Sphingosine, Ethanolamine phosphate, Sphingomyelin, Sphinganine, Sphinganine 1-phosphate, Glucosylceramide, Lactosylceramide, Psychosine, Galactosylceramide, 3-Dehydrosphinganine, Ceramide 1-phosphate, D-Glucosylsphingosine, Globoside, Lactosylceramide sulfate, Sphingosyl-phosphocholine, GM3, alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide, N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, Ceramide 2-aminoethylphosphonate, Ceramide phosphoethanolamine, Sphingosine 1-phosphate, Sulfatide, Digalactosylceramide, Digalactosylceramide sulfate, GM4, GA2, GA1, Dihydroceramide, Phytosphingosine, Phytoceramide, Gangliotriaosylceramide-II3 sulfate
62 M00067_C06125 hsa00600 Sphingolipid metabolism Sphingolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA L-Serine, Palmitoyl-CoA, N-Acylsphingosine, Sphingosine, Ethanolamine phosphate, Sphingomyelin, Sphinganine, Sphinganine 1-phosphate, Glucosylceramide, Lactosylceramide, Psychosine, Galactosylceramide, 3-Dehydrosphinganine, Ceramide 1-phosphate, D-Glucosylsphingosine, Globoside, Lactosylceramide sulfate, Sphingosyl-phosphocholine, GM3, alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide, N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, Ceramide 2-aminoethylphosphonate, Ceramide phosphoethanolamine, Sphingosine 1-phosphate, Sulfatide, Digalactosylceramide, Digalactosylceramide sulfate, GM4, GA2, GA1, Dihydroceramide, Phytosphingosine, Phytoceramide, Gangliotriaosylceramide-II3 sulfate
63 M00067_C06125 hsa00565 Ether lipid metabolism Ether lipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA sn-Glycero-3-phosphocholine, Plasmenylcholine, sn-Glycero-3-phosphoethanolamine, 2-Acetyl-1-alkyl-sn-glycero-3-phosphate, 1-Alkyl-sn-glycerol, 1-Alkyl-2-acylglycerol, Acylglycerone phosphate, 1-Alkenyl-2-acylglycerol, O-Alkylglycerone phosphate, 1-Alkyl-2-acetyl-sn-glycerol, 1-Alkyl-sn-glycero-3-phosphate, 1-Organyl-2-lyso-sn-glycero-3-phosphocholine, 1-O-Alkyl-2-acetyl-3-acyl-sn-glycerol, 1-Alkyl-2-acylglycerophosphoethanolamine, 1-(1-Alkenyl)-sn-glycero-3-phosphocholine, 2-Acetyl-1-alkyl-sn-glycero-3-phosphocholine, 1-(1-Alkenyl)-sn-glycero-3-phosphoethanolamine, O-1-Alk-1-enyl-2-acyl-sn-glycero-3-phosphoethanolamine, 1-Radyl-2-acyl-sn-glycero-3-phosphocholine, 2-Acyl-1-alkyl-sn-glycero-3-phosphate, 1-(1-Alkenyl)-sn-glycerol, 1-(1-Alkenyl)-sn-glycero-3-phosphate, 2-Acyl-1-(1-alkenyl)-sn-glycero-3-phosphate, Seminolipid, Galactosyl-1-alkyl-2-acylglycerol
64 M00067_C20825 hsa00600 Sphingolipid metabolism Sphingolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA L-Serine, Palmitoyl-CoA, N-Acylsphingosine, Sphingosine, Ethanolamine phosphate, Sphingomyelin, Sphinganine, Sphinganine 1-phosphate, Glucosylceramide, Lactosylceramide, Psychosine, Galactosylceramide, 3-Dehydrosphinganine, Ceramide 1-phosphate, D-Glucosylsphingosine, Globoside, Lactosylceramide sulfate, Sphingosyl-phosphocholine, GM3, alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide, N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, Ceramide 2-aminoethylphosphonate, Ceramide phosphoethanolamine, Sphingosine 1-phosphate, Sulfatide, Digalactosylceramide, Digalactosylceramide sulfate, GM4, GA2, GA1, Dihydroceramide, Phytosphingosine, Phytoceramide, Gangliotriaosylceramide-II3 sulfate
65 M00067_C20825 hsa00565 Ether lipid metabolism Ether lipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA sn-Glycero-3-phosphocholine, Plasmenylcholine, sn-Glycero-3-phosphoethanolamine, 2-Acetyl-1-alkyl-sn-glycero-3-phosphate, 1-Alkyl-sn-glycerol, 1-Alkyl-2-acylglycerol, Acylglycerone phosphate, 1-Alkenyl-2-acylglycerol, O-Alkylglycerone phosphate, 1-Alkyl-2-acetyl-sn-glycerol, 1-Alkyl-sn-glycero-3-phosphate, 1-Organyl-2-lyso-sn-glycero-3-phosphocholine, 1-O-Alkyl-2-acetyl-3-acyl-sn-glycerol, 1-Alkyl-2-acylglycerophosphoethanolamine, 1-(1-Alkenyl)-sn-glycero-3-phosphocholine, 2-Acetyl-1-alkyl-sn-glycero-3-phosphocholine, 1-(1-Alkenyl)-sn-glycero-3-phosphoethanolamine, O-1-Alk-1-enyl-2-acyl-sn-glycero-3-phosphoethanolamine, 1-Radyl-2-acyl-sn-glycero-3-phosphocholine, 2-Acyl-1-alkyl-sn-glycero-3-phosphate, 1-(1-Alkenyl)-sn-glycerol, 1-(1-Alkenyl)-sn-glycero-3-phosphate, 2-Acyl-1-(1-alkenyl)-sn-glycero-3-phosphate, Seminolipid, Galactosyl-1-alkyl-2-acylglycerol
66 M00068_G00094 hsa00603 Glycosphingolipid biosynthesis - globo series Glycosphingolipid biosynthesis - globo and isoglobo series - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Lactosylceramide, Globotriaosylceramide, Globoside, Forssman antigen, IV3GalNAcb-Gb4Cer, Galactosylgloboside, Monosialylgalactosylgloboside, Globo-H, V3(NeuAc)2-Gb5Cer, NA G00101, NA G00102, NA G00103, NA G00104, GalGb3Cer, NA G00106, NA G00107, Disialosylglobopentaosylceramide, iGb4-Cer, iGb3-Cer, Globo-A, NA G02977, IV4-alpha-Gal-Gb4Cer, NORint, NOR2 antigen, Globo-B
67 M00069_G00118 hsa00604 Glycosphingolipid biosynthesis - ganglio series Glycosphingolipid biosynthesis - ganglio series - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Lactosylceramide, GM3, GM2, GM1, GD1a, GT1a, GD3, GD2, GD1b, GT1b, GQ1b, GT3, GT2, GT1c, GQ1c, GP1c, GA2, Asialo-GM1, GM1b, GD1c, GD1a, GT1aalpha, GQ1balpha, GM2alpha, GM1alpha, GD1beta, Fucosyl-GM1, N-Acetylgalactosaminyl GM1b, N-Acetylgalactosaminyl GD1a, 9-O-Acetylated GD3, 9-O-Acetylated GT3
68 M00070_G00037 hsa00601 Glycosphingolipid biosynthesis - lacto and neolacto series Glycosphingolipid biosynthesis - lacto and neolacto series - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Lc3Cer, Lc4Cer, NA G00038, Type IB glycolipid, BLeb glycolipid, Type IA glycolipid, ALeb glycolipid, IV2Fuc-Lc4Cer, IV2Fuc,III4Fuc-Lc4Cer, Fuc-Lc4Cer, 3'-isoLM1, Fuc-3'-isoLM1, Paragloboside, IV3Gala-nLc4-Cer, Type II B antigen, Type II A antigen, IV2Fuc-nLc4Cer, III3,IV2Fuc-nLc4Cer, Gal-TypeIIA glycolipid, Type IIIH glycolipid, Type IIIA glycolipid, III3Fuc-nLc4Cer, Sialyl-6-paragloboside, Sialyl-3-paragloboside, IV3NeuAc,III3Fuc-nLc4Cer, 3',8'-LD1, nLc5Cer, nLc6Cer, nLc7Cer, nLc8Cer, VI2Fuc-nLc6, NA G00072, NA G00073, NA G00074, Type IIIAb, III3Fuc-nLc6Cer, NA G00077, iso-nLc8Cer, NA G00079, NA G00080, NA G00081, Trifucosyl Lewis y, NA G00083, NA G00084, NA G00085, NA G00086, VI6NeuAc-nLc6Cer, VI3NeuAc-nLc6Cer, V3Fuc-nLc6Cer, V3Fuc,III3Fuc-nLc6Cer, NA G00091, Lactosylceramide, IV4Gala-nLc4-Cer, III3,IV2Fuca,IV3GalNAca-nLc4-Cer, IV4GalNAcb-SPG ganglioside, PX2, III3,IV2Fuca,IV3Gala-nLc4-Cer
69 M00071_G00050 hsa00601 Glycosphingolipid biosynthesis - lacto and neolacto series Glycosphingolipid biosynthesis - lacto and neolacto series - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Lc3Cer, Lc4Cer, NA G00038, Type IB glycolipid, BLeb glycolipid, Type IA glycolipid, ALeb glycolipid, IV2Fuc-Lc4Cer, IV2Fuc,III4Fuc-Lc4Cer, Fuc-Lc4Cer, 3'-isoLM1, Fuc-3'-isoLM1, Paragloboside, IV3Gala-nLc4-Cer, Type II B antigen, Type II A antigen, IV2Fuc-nLc4Cer, III3,IV2Fuc-nLc4Cer, Gal-TypeIIA glycolipid, Type IIIH glycolipid, Type IIIA glycolipid, III3Fuc-nLc4Cer, Sialyl-6-paragloboside, Sialyl-3-paragloboside, IV3NeuAc,III3Fuc-nLc4Cer, 3',8'-LD1, nLc5Cer, nLc6Cer, nLc7Cer, nLc8Cer, VI2Fuc-nLc6, NA G00072, NA G00073, NA G00074, Type IIIAb, III3Fuc-nLc6Cer, NA G00077, iso-nLc8Cer, NA G00079, NA G00080, NA G00081, Trifucosyl Lewis y, NA G00083, NA G00084, NA G00085, NA G00086, VI6NeuAc-nLc6Cer, VI3NeuAc-nLc6Cer, V3Fuc-nLc6Cer, V3Fuc,III3Fuc-nLc6Cer, NA G00091, Lactosylceramide, IV4Gala-nLc4-Cer, III3,IV2Fuca,IV3GalNAca-nLc4-Cer, IV4GalNAcb-SPG ganglioside, PX2, III3,IV2Fuca,IV3Gala-nLc4-Cer
70 M00073_G00012 hsa00510 N-Glycan biosynthesis N-Glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism N-glycans or asparagine-linked glycans are major constituents of glycoproteins in eukaryotes. N-glycans are covalently attached to asparagine with the consensus sequence of Asn-X-Ser/Thr by an N-glycosidic bond, GlcNAc b1- Asn. Biosynthesis of N-glycans begins on the cytoplasmic face of the ER membrane with the transferase reaction of UDP-GlcNAc and the lipid-like precursor P-Dol (dolichol phosphate) to generate GlcNAc a1- PP-Dol. After sequential addition of monosaccharides by ALG glycosyltransferases [MD:M00055], the N-glycan precursor is attached by the OST (oligosaccharyltransferase) complex to the polypeptide chain that is being synthesized and translocated through the ER membrane. The protein-bound N-glycan precursor is subsequently trimmed, extended, and modified in the ER and Golgi by a complex series of reactions catalyzed by membrane-bound glycosidases and glycosyltransferases. N-glycans thus synthesized are classified into three types: high-mannose type, complex type, and hybrid type. Defects in N-glycan biosynthesis lead to a variety of human diseases known as congenital disorders of glycosylation [DS:H00118 H00119]. GDP-mannose, Dolichyl phosphate, Dolichol, Dolichyl diphosphate, Dolichyl beta-D-glucosyl phosphate, Protein asparagine, Dolichyl phosphate D-mannose, Polyprenol, N-Acetyl-D-glucosaminyldiphosphodolichol, N,N'-Chitobiosyldiphosphodolichol, NA G00003, NA G00004, NA G00005, NA G00006, NA G00007, NA G00008, NA G00009, NA G00010, NA G00011, NA G00012, NA G00013, NA G00014, NA G00015, Core fucose, NA G00017, DS 3, NA G00019, NA G00020, NA G00021, NA G00022, NA G00171, NA G10526, NA G10595, NA G10596, NA G10597, NA G10598, NA G10599, NA G10694
71 M00075_G00019 hsa00510 N-Glycan biosynthesis N-Glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism N-glycans or asparagine-linked glycans are major constituents of glycoproteins in eukaryotes. N-glycans are covalently attached to asparagine with the consensus sequence of Asn-X-Ser/Thr by an N-glycosidic bond, GlcNAc b1- Asn. Biosynthesis of N-glycans begins on the cytoplasmic face of the ER membrane with the transferase reaction of UDP-GlcNAc and the lipid-like precursor P-Dol (dolichol phosphate) to generate GlcNAc a1- PP-Dol. After sequential addition of monosaccharides by ALG glycosyltransferases [MD:M00055], the N-glycan precursor is attached by the OST (oligosaccharyltransferase) complex to the polypeptide chain that is being synthesized and translocated through the ER membrane. The protein-bound N-glycan precursor is subsequently trimmed, extended, and modified in the ER and Golgi by a complex series of reactions catalyzed by membrane-bound glycosidases and glycosyltransferases. N-glycans thus synthesized are classified into three types: high-mannose type, complex type, and hybrid type. Defects in N-glycan biosynthesis lead to a variety of human diseases known as congenital disorders of glycosylation [DS:H00118 H00119]. GDP-mannose, Dolichyl phosphate, Dolichol, Dolichyl diphosphate, Dolichyl beta-D-glucosyl phosphate, Protein asparagine, Dolichyl phosphate D-mannose, Polyprenol, N-Acetyl-D-glucosaminyldiphosphodolichol, N,N'-Chitobiosyldiphosphodolichol, NA G00003, NA G00004, NA G00005, NA G00006, NA G00007, NA G00008, NA G00009, NA G00010, NA G00011, NA G00012, NA G00013, NA G00014, NA G00015, Core fucose, NA G00017, DS 3, NA G00019, NA G00020, NA G00021, NA G00022, NA G00171, NA G10526, NA G10595, NA G10596, NA G10597, NA G10598, NA G10599, NA G10694
72 M00075_G00022 hsa00510 N-Glycan biosynthesis N-Glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism N-glycans or asparagine-linked glycans are major constituents of glycoproteins in eukaryotes. N-glycans are covalently attached to asparagine with the consensus sequence of Asn-X-Ser/Thr by an N-glycosidic bond, GlcNAc b1- Asn. Biosynthesis of N-glycans begins on the cytoplasmic face of the ER membrane with the transferase reaction of UDP-GlcNAc and the lipid-like precursor P-Dol (dolichol phosphate) to generate GlcNAc a1- PP-Dol. After sequential addition of monosaccharides by ALG glycosyltransferases [MD:M00055], the N-glycan precursor is attached by the OST (oligosaccharyltransferase) complex to the polypeptide chain that is being synthesized and translocated through the ER membrane. The protein-bound N-glycan precursor is subsequently trimmed, extended, and modified in the ER and Golgi by a complex series of reactions catalyzed by membrane-bound glycosidases and glycosyltransferases. N-glycans thus synthesized are classified into three types: high-mannose type, complex type, and hybrid type. Defects in N-glycan biosynthesis lead to a variety of human diseases known as congenital disorders of glycosylation [DS:H00118 H00119]. GDP-mannose, Dolichyl phosphate, Dolichol, Dolichyl diphosphate, Dolichyl beta-D-glucosyl phosphate, Protein asparagine, Dolichyl phosphate D-mannose, Polyprenol, N-Acetyl-D-glucosaminyldiphosphodolichol, N,N'-Chitobiosyldiphosphodolichol, NA G00003, NA G00004, NA G00005, NA G00006, NA G00007, NA G00008, NA G00009, NA G00010, NA G00011, NA G00012, NA G00013, NA G00014, NA G00015, Core fucose, NA G00017, DS 3, NA G00019, NA G00020, NA G00021, NA G00022, NA G00171, NA G10526, NA G10595, NA G10596, NA G10597, NA G10598, NA G10599, NA G10694
73 M00075_G00018 hsa00510 N-Glycan biosynthesis N-Glycan biosynthesis - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism N-glycans or asparagine-linked glycans are major constituents of glycoproteins in eukaryotes. N-glycans are covalently attached to asparagine with the consensus sequence of Asn-X-Ser/Thr by an N-glycosidic bond, GlcNAc b1- Asn. Biosynthesis of N-glycans begins on the cytoplasmic face of the ER membrane with the transferase reaction of UDP-GlcNAc and the lipid-like precursor P-Dol (dolichol phosphate) to generate GlcNAc a1- PP-Dol. After sequential addition of monosaccharides by ALG glycosyltransferases [MD:M00055], the N-glycan precursor is attached by the OST (oligosaccharyltransferase) complex to the polypeptide chain that is being synthesized and translocated through the ER membrane. The protein-bound N-glycan precursor is subsequently trimmed, extended, and modified in the ER and Golgi by a complex series of reactions catalyzed by membrane-bound glycosidases and glycosyltransferases. N-glycans thus synthesized are classified into three types: high-mannose type, complex type, and hybrid type. Defects in N-glycan biosynthesis lead to a variety of human diseases known as congenital disorders of glycosylation [DS:H00118 H00119]. GDP-mannose, Dolichyl phosphate, Dolichol, Dolichyl diphosphate, Dolichyl beta-D-glucosyl phosphate, Protein asparagine, Dolichyl phosphate D-mannose, Polyprenol, N-Acetyl-D-glucosaminyldiphosphodolichol, N,N'-Chitobiosyldiphosphodolichol, NA G00003, NA G00004, NA G00005, NA G00006, NA G00007, NA G00008, NA G00009, NA G00010, NA G00011, NA G00012, NA G00013, NA G00014, NA G00015, Core fucose, NA G00017, DS 3, NA G00019, NA G00020, NA G00021, NA G00022, NA G00171, NA G10526, NA G10595, NA G10596, NA G10597, NA G10598, NA G10599, NA G10694
74 M00076_G00872 hsa00531 Glycosaminoglycan degradation Glycosaminoglycan degradation - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Dermatan sulfate, Keratan sulfate, Chondroitin 4-sulfate, Heparan sulfate, NA G00526, NA G00711, DWA-2, NA G01391, NA G01945, NA G01977, NA G02632, NA G09660, NA G10008, Hyaluronic acid, NA G12336, NA G13034, NA G13035, NA G13036, NA G13037, NA G13038, NA G13039, NA G13040, NA G13041, NA G13042, NA G13043, NA G13073, NA G13074
75 M00077_G00872 hsa00531 Glycosaminoglycan degradation Glycosaminoglycan degradation - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Dermatan sulfate, Keratan sulfate, Chondroitin 4-sulfate, Heparan sulfate, NA G00526, NA G00711, DWA-2, NA G01391, NA G01945, NA G01977, NA G02632, NA G09660, NA G10008, Hyaluronic acid, NA G12336, NA G13034, NA G13035, NA G13036, NA G13037, NA G13038, NA G13039, NA G13040, NA G13041, NA G13042, NA G13043, NA G13073, NA G13074
76 M00078_G02632 hsa00531 Glycosaminoglycan degradation Glycosaminoglycan degradation - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Dermatan sulfate, Keratan sulfate, Chondroitin 4-sulfate, Heparan sulfate, NA G00526, NA G00711, DWA-2, NA G01391, NA G01945, NA G01977, NA G02632, NA G09660, NA G10008, Hyaluronic acid, NA G12336, NA G13034, NA G13035, NA G13036, NA G13037, NA G13038, NA G13039, NA G13040, NA G13041, NA G13042, NA G13043, NA G13073, NA G13074
77 M00079_G01391 hsa00531 Glycosaminoglycan degradation Glycosaminoglycan degradation - Homo sapiens (human) Metabolism; Glycan biosynthesis and metabolism NA Dermatan sulfate, Keratan sulfate, Chondroitin 4-sulfate, Heparan sulfate, NA G00526, NA G00711, DWA-2, NA G01391, NA G01945, NA G01977, NA G02632, NA G09660, NA G10008, Hyaluronic acid, NA G12336, NA G13034, NA G13035, NA G13036, NA G13037, NA G13038, NA G13039, NA G13040, NA G13041, NA G13042, NA G13043, NA G13073, NA G13074
78 M00082_C05744 hsa01212 Fatty acid metabolism Fatty acid metabolism - Homo sapiens (human) NA NA NA
79 M00082_C05744 hsa00061 Fatty acid biosynthesis Fatty acid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism NA Acetyl-CoA, Malonyl-CoA, Palmitoyl-CoA, Acyl-[acyl-carrier protein], Acyl-carrier protein, Hexadecanoic acid, Malonate, Long-chain fatty acid, 3-Oxoacyl-[acyl-carrier protein], trans-2,3-Dehydroacyl-[acyl-carrier protein], (9Z)-Octadecenoic acid, Oleoyl-[acyl-carrier protein], Malonyl-[acyl-carrier protein], (3R)-3-Hydroxyacyl-[acyl-carrier protein], Octadecanoic acid, Decanoic acid, Dodecanoic acid, Long-chain acyl-CoA, Acetyl-[acyl-carrier protein], Octadecanoyl-[acyl-carrier protein], cis-Dec-3-enoyl-[acp], But-2-enoyl-[acyl-carrier protein], (3R)-3-Hydroxybutanoyl-[acyl-carrier protein], (3R)-3-Hydroxydecanoyl-[acyl-carrier protein], (3R)-3-Hydroxyoctanoyl-[acyl-carrier protein], (3R)-3-Hydroxypalmitoyl-[acyl-carrier protein], (3R)-3-Hydroxytetradecanoyl-[acyl-carrier protein], Dodecanoyl-[acyl-carrier protein], Acetoacetyl-[acp], Butyryl-[acp], 3-Oxohexanoyl-[acp], (3R)-3-Hydroxyhexanoyl-[acyl-carrier protein], trans-Hex-2-enoyl-[acp], Hexanoyl-[acp], 3-Oxooctanoyl-[acp], trans-Oct-2-enoyl-[acp], Octanoyl-[acp], 3-Oxodecanoyl-[acp], trans-Dec-2-enoyl-[acp], Decanoyl-[acp], 3-Oxododecanoyl-[acp], (3R)-3-Hydroxydodecanoyl-[acyl-carrier protein], trans-Dodec-2-enoyl-[acp], 3-Oxotetradecanoyl-[acp], trans-Tetradec-2-enoyl-[acp], Tetradecanoyl-[acp], 3-Oxohexadecanoyl-[acp], trans-Hexadec-2-enoyl-[acp], Hexadecanoyl-[acp], Octanoic acid, Tetradecanoic acid, (9Z)-Hexadecenoic acid, 3-Oxostearoyl-[acp], (3R)-3-Hydroxyoctadecanoyl-[acyl-carrier protein], (2E)-Octadecenoyl-[acp], Hexadecenoyl-[acyl-carrier protein], Long-chain acyl-[acyl-carrier protein], n-7 Unsaturated acyl-[acyl-carrier protein]
80 M00083_C05745 hsa01212 Fatty acid metabolism Fatty acid metabolism - Homo sapiens (human) NA NA NA
81 M00083_C05745 hsa00061 Fatty acid biosynthesis Fatty acid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism NA Acetyl-CoA, Malonyl-CoA, Palmitoyl-CoA, Acyl-[acyl-carrier protein], Acyl-carrier protein, Hexadecanoic acid, Malonate, Long-chain fatty acid, 3-Oxoacyl-[acyl-carrier protein], trans-2,3-Dehydroacyl-[acyl-carrier protein], (9Z)-Octadecenoic acid, Oleoyl-[acyl-carrier protein], Malonyl-[acyl-carrier protein], (3R)-3-Hydroxyacyl-[acyl-carrier protein], Octadecanoic acid, Decanoic acid, Dodecanoic acid, Long-chain acyl-CoA, Acetyl-[acyl-carrier protein], Octadecanoyl-[acyl-carrier protein], cis-Dec-3-enoyl-[acp], But-2-enoyl-[acyl-carrier protein], (3R)-3-Hydroxybutanoyl-[acyl-carrier protein], (3R)-3-Hydroxydecanoyl-[acyl-carrier protein], (3R)-3-Hydroxyoctanoyl-[acyl-carrier protein], (3R)-3-Hydroxypalmitoyl-[acyl-carrier protein], (3R)-3-Hydroxytetradecanoyl-[acyl-carrier protein], Dodecanoyl-[acyl-carrier protein], Acetoacetyl-[acp], Butyryl-[acp], 3-Oxohexanoyl-[acp], (3R)-3-Hydroxyhexanoyl-[acyl-carrier protein], trans-Hex-2-enoyl-[acp], Hexanoyl-[acp], 3-Oxooctanoyl-[acp], trans-Oct-2-enoyl-[acp], Octanoyl-[acp], 3-Oxodecanoyl-[acp], trans-Dec-2-enoyl-[acp], Decanoyl-[acp], 3-Oxododecanoyl-[acp], (3R)-3-Hydroxydodecanoyl-[acyl-carrier protein], trans-Dodec-2-enoyl-[acp], 3-Oxotetradecanoyl-[acp], trans-Tetradec-2-enoyl-[acp], Tetradecanoyl-[acp], 3-Oxohexadecanoyl-[acp], trans-Hexadec-2-enoyl-[acp], Hexadecanoyl-[acp], Octanoic acid, Tetradecanoic acid, (9Z)-Hexadecenoic acid, 3-Oxostearoyl-[acp], (3R)-3-Hydroxyoctadecanoyl-[acyl-carrier protein], (2E)-Octadecenoyl-[acp], Hexadecenoyl-[acyl-carrier protein], Long-chain acyl-[acyl-carrier protein], n-7 Unsaturated acyl-[acyl-carrier protein]
82 M00085_C00040 hsa01212 Fatty acid metabolism Fatty acid metabolism - Homo sapiens (human) NA NA NA
83 M00085_C00040 hsa00062 Fatty acid elongation Fatty acid elongation - Homo sapiens (human) Metabolism; Lipid metabolism NA Acetyl-CoA, Acyl-CoA, Malonyl-CoA, Butanoyl-CoA, Palmitoyl-CoA, Fatty acid, Hexadecanoic acid, 3-Oxoacyl-CoA, 2,3-Dehydroacyl-CoA, Long-chain fatty acid, (3S)-3-Hydroxyacyl-CoA, trans-2,3-Dehydroacyl-CoA, Lauroyl-CoA, Octanoyl-CoA, Tetradecanoyl-CoA, Long-chain acyl-CoA, 2-trans-Dodecenoyl-CoA, (S)-3-Hydroxyhexadecanoyl-CoA, 3-Oxopalmitoyl-CoA, (S)-3-Hydroxytetradecanoyl-CoA, 3-Oxotetradecanoyl-CoA, (S)-3-Hydroxydodecanoyl-CoA, 3-Oxododecanoyl-CoA, (S)-Hydroxydecanoyl-CoA, 3-Oxodecanoyl-CoA, (S)-3-Hydroxyoctanoyl-CoA, 3-Oxooctanoyl-CoA, (S)-Hydroxyhexanoyl-CoA, 3-Oxohexanoyl-CoA, Hexanoyl-CoA, trans-Hex-2-enoyl-CoA, trans-Hexadec-2-enoyl-CoA, trans-Tetradec-2-enoyl-CoA, Decanoyl-CoA, trans-Dec-2-enoyl-CoA, trans-Oct-2-enoyl-CoA, Very-long-chain acyl-CoA, Very-long-chain 3-oxoacyl-CoA, Very-long-chain (3R)-3-hydroxyacyl-CoA, Very-long-chain trans-2,3-dehydroacyl-CoA
84 M00087_C02593 hsa01212 Fatty acid metabolism Fatty acid metabolism - Homo sapiens (human) NA NA NA
85 M00087_C02593 hsa00071 Fatty acid metabolism Fatty acid degradation - Homo sapiens (human) Metabolism; Lipid metabolism NA CoA, Acetyl-CoA, Aldehyde, Butanoyl-CoA, Palmitoyl-CoA, Fatty acid, Primary alcohol, Acyl-carrier protein, Hexadecanoic acid, Acetoacetyl-CoA, Reduced rubredoxin, Oxidized rubredoxin, Glutarate, Hexadecanal, Glutaryl-CoA, Long-chain fatty acid, 1-Hexadecanol, Crotonoyl-CoA, (S)-3-Hydroxybutanoyl-CoA, Alkane, Lauroyl-CoA, Octanoyl-CoA, Tetradecanoyl-CoA, L-Palmitoylcarnitine, 2-trans-Dodecenoyl-CoA, omega-Hydroxy fatty acid, (R)-3-Hydroxybutanoyl-CoA, alpha-Hydroxy fatty acid, (S)-3-Hydroxyhexadecanoyl-CoA, 3-Oxopalmitoyl-CoA, (S)-3-Hydroxytetradecanoyl-CoA, 3-Oxotetradecanoyl-CoA, (S)-3-Hydroxydodecanoyl-CoA, 3-Oxododecanoyl-CoA, (S)-Hydroxydecanoyl-CoA, 3-Oxodecanoyl-CoA, (S)-3-Hydroxyoctanoyl-CoA, 3-Oxooctanoyl-CoA, (S)-Hydroxyhexanoyl-CoA, 3-Oxohexanoyl-CoA, Hexanoyl-CoA, trans-Hex-2-enoyl-CoA, trans-Hexadec-2-enoyl-CoA, trans-Tetradec-2-enoyl-CoA, Decanoyl-CoA, trans-Dec-2-enoyl-CoA, trans-Oct-2-enoyl-CoA, trans,cis-Lauro-2,6-dienoyl-CoA, cis,cis-3,6-Dodecadienoyl-CoA, Long-chain acyl-[acyl-carrier protein]
86 M00089_C00422 hsa00561 Glycerolipid metabolism Glycerolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA UDP-glucose, Acyl-CoA, sn-Glycerol 3-phosphate, D-Glucose 1-phosphate, Glycerone phosphate, Glycerol, Fatty acid, Acyl-[acyl-carrier protein], Glycerone, 3-Phospho-D-glycerate, D-Glycerate, Phosphatidate, Triacylglycerol, D-Glyceraldehyde, 2-Phospho-D-glycerate, 1,2-Diacyl-sn-glycerol, 1-Acyl-sn-glycerol 3-phosphate, 3-Hydroxypropanal, 1-Acylglycerol, Acyl phosphate, Propane-1,3-diol, 1,2-Diacyl-3-beta-D-galactosyl-sn-glycerol, 3-D-Glucosyl-1,2-diacylglycerol, 2-Acyl-3-O-(beta-D-galactosyl)-sn-glycerol, 1,2-Diacyl-3-[3-(alpha-D-N-acetylneuraminyl)-beta-D-galactosyl]-sn-glycerol, 3-beta-D-Galactosyl-sn-glycerol, Digalactosyl-diacylglycerol, Acyl1-monogalactosyl-diacylglycerol, Diglucosyldiacylglycerol, Glycerophosphoglycoglycerolipid, Lipoteichoic acid, 1,2-Diacyl-3-alpha-D-glucosyl-sn-glycerol, alpha-Kojibiosyldiacylglycerol, UDP-6-sulfoquinovose, Sulfoquinovosyldiacylglycerol, Glycerophosphoglycoglycerolipid, Lipoteichoic acid, 1,2-Diacyl-3-O-[beta-D-galactosyl-(1->6)-beta-D-galactosyl]-sn-glycerol
87 M00090_C00157 hsa00564 Glycerophospholipid metabolism Glycerophospholipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA Acyl-CoA, L-Serine, Acetaldehyde, sn-Glycerol 3-phosphate, Glycerone phosphate, Choline, Phosphatidylcholine, Ethanolamine, CDP-diacylglycerol, CDP-choline, Phosphatidylglycerol, Ethanolamine phosphate, Phosphatidylethanolamine, Phosphatidate, CDP-glycerol, CDP-ethanolamine, Choline phosphate, sn-Glycerol 1-phosphate, 1,2-Diacyl-sn-glycerol, sn-Glycero-3-phosphocholine, 1-Acyl-sn-glycerol 3-phosphate, 1-Phosphatidyl-D-myo-inositol, N-Methylethanolamine phosphate, sn-Glycero-3-phosphoethanolamine, Phosphatidyl-N-methylethanolamine, Acetylcholine, Phosphatidylserine, Acylglycerone phosphate, 1-Acylglycerophosphoinositol, L-Serine-phosphoethanolamine, Phosphatidylglycerophosphate, 2-Acyl-sn-glycerol 3-phosphate, 1-Acyl-sn-glycero-3-phosphocholine, 2-Acyl-sn-glycero-3-phosphocholine, Phosphatidyl-N-dimethylethanolamine, 1-Acyl-sn-glycero-3-phosphoethanolamine, 3-(O-Geranylgeranyl)-sn-glycerol 1-phosphate, 2,3-Bis-(O-geranylgeranyl)-sn-glycerol 1-phosphate, 2-Acyl-sn-glycero-3-phosphoethanolamine, 2-Acyl-sn-glycero-3-phosphoserine, Aminoacyl-phosphatidylglycerol, Cardiolipin, Triethanolamine, Diethanolamine, Phosphodimethylethanolamine, CDP-2,3-bis-(O-geranylgeranyl)-sn-glycerol, 1-Acyl-sn-glycero-3-phosphoserine, 1-Acyl-sn-glycero-3-phosphoglycerol, Monolysocardiolipin, Diacylglycerylhomoserine, Diacylglyceryl-N,N,N-trimethylhomoserine, 2,3-Bis-(O-geranylgeranyl)-sn-glycero-1-phospho-L-serine, 2,3-Bis-(O-phytanyl)-sn-glycerol 1-phosphate, Heptaprenylglyceryl phosphate, Heptaprenylglycerol, Diacetylheptaprenylglycerol
88 M00091_C00157 hsa00564 Glycerophospholipid metabolism Glycerophospholipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA Acyl-CoA, L-Serine, Acetaldehyde, sn-Glycerol 3-phosphate, Glycerone phosphate, Choline, Phosphatidylcholine, Ethanolamine, CDP-diacylglycerol, CDP-choline, Phosphatidylglycerol, Ethanolamine phosphate, Phosphatidylethanolamine, Phosphatidate, CDP-glycerol, CDP-ethanolamine, Choline phosphate, sn-Glycerol 1-phosphate, 1,2-Diacyl-sn-glycerol, sn-Glycero-3-phosphocholine, 1-Acyl-sn-glycerol 3-phosphate, 1-Phosphatidyl-D-myo-inositol, N-Methylethanolamine phosphate, sn-Glycero-3-phosphoethanolamine, Phosphatidyl-N-methylethanolamine, Acetylcholine, Phosphatidylserine, Acylglycerone phosphate, 1-Acylglycerophosphoinositol, L-Serine-phosphoethanolamine, Phosphatidylglycerophosphate, 2-Acyl-sn-glycerol 3-phosphate, 1-Acyl-sn-glycero-3-phosphocholine, 2-Acyl-sn-glycero-3-phosphocholine, Phosphatidyl-N-dimethylethanolamine, 1-Acyl-sn-glycero-3-phosphoethanolamine, 3-(O-Geranylgeranyl)-sn-glycerol 1-phosphate, 2,3-Bis-(O-geranylgeranyl)-sn-glycerol 1-phosphate, 2-Acyl-sn-glycero-3-phosphoethanolamine, 2-Acyl-sn-glycero-3-phosphoserine, Aminoacyl-phosphatidylglycerol, Cardiolipin, Triethanolamine, Diethanolamine, Phosphodimethylethanolamine, CDP-2,3-bis-(O-geranylgeranyl)-sn-glycerol, 1-Acyl-sn-glycero-3-phosphoserine, 1-Acyl-sn-glycero-3-phosphoglycerol, Monolysocardiolipin, Diacylglycerylhomoserine, Diacylglyceryl-N,N,N-trimethylhomoserine, 2,3-Bis-(O-geranylgeranyl)-sn-glycero-1-phospho-L-serine, 2,3-Bis-(O-phytanyl)-sn-glycerol 1-phosphate, Heptaprenylglyceryl phosphate, Heptaprenylglycerol, Diacetylheptaprenylglycerol
89 M00092_C00350 hsa00564 Glycerophospholipid metabolism Glycerophospholipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA Acyl-CoA, L-Serine, Acetaldehyde, sn-Glycerol 3-phosphate, Glycerone phosphate, Choline, Phosphatidylcholine, Ethanolamine, CDP-diacylglycerol, CDP-choline, Phosphatidylglycerol, Ethanolamine phosphate, Phosphatidylethanolamine, Phosphatidate, CDP-glycerol, CDP-ethanolamine, Choline phosphate, sn-Glycerol 1-phosphate, 1,2-Diacyl-sn-glycerol, sn-Glycero-3-phosphocholine, 1-Acyl-sn-glycerol 3-phosphate, 1-Phosphatidyl-D-myo-inositol, N-Methylethanolamine phosphate, sn-Glycero-3-phosphoethanolamine, Phosphatidyl-N-methylethanolamine, Acetylcholine, Phosphatidylserine, Acylglycerone phosphate, 1-Acylglycerophosphoinositol, L-Serine-phosphoethanolamine, Phosphatidylglycerophosphate, 2-Acyl-sn-glycerol 3-phosphate, 1-Acyl-sn-glycero-3-phosphocholine, 2-Acyl-sn-glycero-3-phosphocholine, Phosphatidyl-N-dimethylethanolamine, 1-Acyl-sn-glycero-3-phosphoethanolamine, 3-(O-Geranylgeranyl)-sn-glycerol 1-phosphate, 2,3-Bis-(O-geranylgeranyl)-sn-glycerol 1-phosphate, 2-Acyl-sn-glycero-3-phosphoethanolamine, 2-Acyl-sn-glycero-3-phosphoserine, Aminoacyl-phosphatidylglycerol, Cardiolipin, Triethanolamine, Diethanolamine, Phosphodimethylethanolamine, CDP-2,3-bis-(O-geranylgeranyl)-sn-glycerol, 1-Acyl-sn-glycero-3-phosphoserine, 1-Acyl-sn-glycero-3-phosphoglycerol, Monolysocardiolipin, Diacylglycerylhomoserine, Diacylglyceryl-N,N,N-trimethylhomoserine, 2,3-Bis-(O-geranylgeranyl)-sn-glycero-1-phospho-L-serine, 2,3-Bis-(O-phytanyl)-sn-glycerol 1-phosphate, Heptaprenylglyceryl phosphate, Heptaprenylglycerol, Diacetylheptaprenylglycerol
90 M00094_C00195 hsa00600 Sphingolipid metabolism Sphingolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA L-Serine, Palmitoyl-CoA, N-Acylsphingosine, Sphingosine, Ethanolamine phosphate, Sphingomyelin, Sphinganine, Sphinganine 1-phosphate, Glucosylceramide, Lactosylceramide, Psychosine, Galactosylceramide, 3-Dehydrosphinganine, Ceramide 1-phosphate, D-Glucosylsphingosine, Globoside, Lactosylceramide sulfate, Sphingosyl-phosphocholine, GM3, alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide, N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, Ceramide 2-aminoethylphosphonate, Ceramide phosphoethanolamine, Sphingosine 1-phosphate, Sulfatide, Digalactosylceramide, Digalactosylceramide sulfate, GM4, GA2, GA1, Dihydroceramide, Phytosphingosine, Phytoceramide, Gangliotriaosylceramide-II3 sulfate
91 M00095_C00235 hsa00900 Terpenoid backbone biosynthesis Terpenoid backbone biosynthesis - Homo sapiens (human) Metabolism; Metabolism of terpenoids and polyketides Terpenoids, also known as isoprenoids, are a large class of natural products consisting of isoprene (C5) units. There are two biosynthetic pathways, the mevalonate pathway [MD:M00095] and the non-mevalonate pathway or the MEP/DOXP pathway [MD:M00096], for the terpenoid building blocks: isopentenyl diphosphate (IPP) and dimethylallyl diphosphate (DMAPP). The action of prenyltransferases then generates higher-order building blocks: geranyl diphosphate (GPP), farsenyl diphosphate (FPP), and geranylgeranyl diphosphate (GGPP), which are the precursors of monoterpenoids (C10), sesquiterpenoids (C15), and diterpenoids (C20), respectively. Condensation of these building blocks gives rise to the precursors of sterols (C30) and carotenoids (C40). The MEP/DOXP pathway is absent in higher animals and fungi, but in green plants the MEP/DOXP and mevalonate pathways co-exist in separate cellular compartments. The MEP/DOXP pathway, operating in the plastids, is responsible for the formation of essential oil monoterpenes and linalyl acetate, some sesquiterpenes, diterpenes, and carotenoids and phytol. The mevalonate pathway, operating in the cytosol, gives rise to triterpenes, sterols, and most sesquiterpenes. Pyruvate, Acetyl-CoA, D-Glyceraldehyde 3-phosphate, Isopentenyl diphosphate, Dimethylallyl diphosphate, Acetoacetyl-CoA, Geranyl diphosphate, Geranylgeranyl diphosphate, (S)-3-Hydroxy-3-methylglutaryl-CoA, (R)-Mevalonate, trans,trans-Farnesyl diphosphate, (R)-5-Phosphomevalonate, (2E,6E)-Farnesol, (R)-5-Diphosphomevalonate, all-trans-Hexaprenyl diphosphate, cis-1,4-Polyisoprene, 2-trans,6-trans-Farnesal, all-trans-Nonaprenyl diphosphate, all-trans-Octaprenyl diphosphate, all-trans-Heptaprenyl diphosphate, Protein C-terminal S-farnesyl-L-cysteine, di-trans,poly-cis-Undecaprenyl diphosphate, Protein C-terminal S-farnesyl-L-cysteine methyl ester, Phytyl diphosphate, ditrans,polycis-Polyprenyl diphosphate, Polyprenol, 2-C-Methyl-D-erythritol 4-phosphate, 4-(Cytidine 5'-diphospho)-2-C-methyl-D-erythritol, 2-Phospho-4-(cytidine 5'-diphospho)-2-C-methyl-D-erythritol, 1-Deoxy-D-xylulose 5-phosphate, 2-C-Methyl-D-erythritol 2,4-cyclodiphosphate, 1-Hydroxy-2-methyl-2-butenyl 4-diphosphate, Isoprene, 2-cis,6-trans-Farnesyl diphosphate, all-trans-Decaprenyl diphosphate, trans,octacis-Decaprenyl diphosphate, Farnesylcysteine, 2-cis,6-cis-Farnesyl diphosphate, tritrans,heptacis-Undecaprenyl diphosphate, trans,polycis-Polyprenyl diphosphate, S-Farnesyl protein, trans,trans-Farnesyl phosphate, Isopentenyl phosphate, (R)-Mevalonate 3-phosphate, (R)-Mevalonate 3,5-bisphosphate, trans-Anhydromevalonate 5-phosphate
92 M00098_C00116 hsa00561 Glycerolipid metabolism Glycerolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA UDP-glucose, Acyl-CoA, sn-Glycerol 3-phosphate, D-Glucose 1-phosphate, Glycerone phosphate, Glycerol, Fatty acid, Acyl-[acyl-carrier protein], Glycerone, 3-Phospho-D-glycerate, D-Glycerate, Phosphatidate, Triacylglycerol, D-Glyceraldehyde, 2-Phospho-D-glycerate, 1,2-Diacyl-sn-glycerol, 1-Acyl-sn-glycerol 3-phosphate, 3-Hydroxypropanal, 1-Acylglycerol, Acyl phosphate, Propane-1,3-diol, 1,2-Diacyl-3-beta-D-galactosyl-sn-glycerol, 3-D-Glucosyl-1,2-diacylglycerol, 2-Acyl-3-O-(beta-D-galactosyl)-sn-glycerol, 1,2-Diacyl-3-[3-(alpha-D-N-acetylneuraminyl)-beta-D-galactosyl]-sn-glycerol, 3-beta-D-Galactosyl-sn-glycerol, Digalactosyl-diacylglycerol, Acyl1-monogalactosyl-diacylglycerol, Diglucosyldiacylglycerol, Glycerophosphoglycoglycerolipid, Lipoteichoic acid, 1,2-Diacyl-3-alpha-D-glucosyl-sn-glycerol, alpha-Kojibiosyldiacylglycerol, UDP-6-sulfoquinovose, Sulfoquinovosyldiacylglycerol, Glycerophosphoglycoglycerolipid, Lipoteichoic acid, 1,2-Diacyl-3-O-[beta-D-galactosyl-(1->6)-beta-D-galactosyl]-sn-glycerol
93 M00099_C00319 hsa00600 Sphingolipid metabolism Sphingolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA L-Serine, Palmitoyl-CoA, N-Acylsphingosine, Sphingosine, Ethanolamine phosphate, Sphingomyelin, Sphinganine, Sphinganine 1-phosphate, Glucosylceramide, Lactosylceramide, Psychosine, Galactosylceramide, 3-Dehydrosphinganine, Ceramide 1-phosphate, D-Glucosylsphingosine, Globoside, Lactosylceramide sulfate, Sphingosyl-phosphocholine, GM3, alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide, N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, Ceramide 2-aminoethylphosphonate, Ceramide phosphoethanolamine, Sphingosine 1-phosphate, Sulfatide, Digalactosylceramide, Digalactosylceramide sulfate, GM4, GA2, GA1, Dihydroceramide, Phytosphingosine, Phytoceramide, Gangliotriaosylceramide-II3 sulfate
94 M00100_C00346 hsa00600 Sphingolipid metabolism Sphingolipid metabolism - Homo sapiens (human) Metabolism; Lipid metabolism NA L-Serine, Palmitoyl-CoA, N-Acylsphingosine, Sphingosine, Ethanolamine phosphate, Sphingomyelin, Sphinganine, Sphinganine 1-phosphate, Glucosylceramide, Lactosylceramide, Psychosine, Galactosylceramide, 3-Dehydrosphinganine, Ceramide 1-phosphate, D-Glucosylsphingosine, Globoside, Lactosylceramide sulfate, Sphingosyl-phosphocholine, GM3, alpha-D-Galactosyl-(1->4)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1)-ceramide, N-Acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, D-Galactosyl-N-acetyl-D-galactosaminyl-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide, Ceramide 2-aminoethylphosphonate, Ceramide phosphoethanolamine, Sphingosine 1-phosphate, Sulfatide, Digalactosylceramide, Digalactosylceramide sulfate, GM4, GA2, GA1, Dihydroceramide, Phytosphingosine, Phytoceramide, Gangliotriaosylceramide-II3 sulfate
95 M00101_C00187 hsa00100 Steroid biosynthesis Steroid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism NA Cholesterol, trans,trans-Farnesyl diphosphate, Squalene, (S)-2,3-Epoxysqualene, 7-Dehydrocholesterol, Lathosterol, Calcidiol, Calcitriol, Ergosterol, Lanosterol, beta-Sitosterol, Campesterol, Desmosterol, Cycloartenol, Obtusifoliol, Cycloeucalenol, Cholesterol ester, Presqualene diphosphate, 5alpha-Cholest-8-en-3beta-ol, Fecosterol, 4alpha-Methylzymosterol, 7-Dehydrodesmosterol, 14-Demethyllanosterol, 24,25-Dihydrolanosterol, Zymosterol, 5alpha-Cholesta-7,24-dien-3beta-ol, Ergosta-5,7,22,24(28)-tetraen-3beta-ol, Vitamin D2, Stigmasterol, Vitamin D3, Secalciferol, Brassicasterol, Isofucosterol, 24-Methylidenecycloartanol, 4,4-Dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol, 4alpha-Methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol, 24-Methylenelophenol, 24-Ethylidenelophenol, 4alpha-Methylfecosterol, Episterol, 5-Dehydroepisterol, 24-Methylenecholesterol, Delta7-Avenasterol, 5-Dehydroavenasterol, 4alpha-Methylzymosterol-4-carboxylate, 3-Keto-4-methylzymosterol, 4,4-Dimethyl-5alpha-cholesta-8-en-3beta-ol, Calcitetrol, 24-epi-Campesterol, 4alpha-Carboxy-5alpha-cholesta-8,24-dien-3beta-ol, 3beta-Hydroxy-4beta,14alpha-dimethyl-9beta,19-cyclo-5alpha-ergost-24(24(1))-en-4alpha-carboxylate, 3beta-Hydroxyergosta-7,24(24(1))-dien-4alpha-carboxylate, 4alpha-Carboxy-stigmasta-7,24(24(1))-dien-3beta-ol, Cycloeucalenone, Episterone, Avenastenone, Zymosterone
96 M00103_C01673 hsa00100 Steroid biosynthesis Steroid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism NA Cholesterol, trans,trans-Farnesyl diphosphate, Squalene, (S)-2,3-Epoxysqualene, 7-Dehydrocholesterol, Lathosterol, Calcidiol, Calcitriol, Ergosterol, Lanosterol, beta-Sitosterol, Campesterol, Desmosterol, Cycloartenol, Obtusifoliol, Cycloeucalenol, Cholesterol ester, Presqualene diphosphate, 5alpha-Cholest-8-en-3beta-ol, Fecosterol, 4alpha-Methylzymosterol, 7-Dehydrodesmosterol, 14-Demethyllanosterol, 24,25-Dihydrolanosterol, Zymosterol, 5alpha-Cholesta-7,24-dien-3beta-ol, Ergosta-5,7,22,24(28)-tetraen-3beta-ol, Vitamin D2, Stigmasterol, Vitamin D3, Secalciferol, Brassicasterol, Isofucosterol, 24-Methylidenecycloartanol, 4,4-Dimethyl-5alpha-cholesta-8,14,24-trien-3beta-ol, 4alpha-Methyl-5alpha-ergosta-8,14,24(28)-trien-3beta-ol, 24-Methylenelophenol, 24-Ethylidenelophenol, 4alpha-Methylfecosterol, Episterol, 5-Dehydroepisterol, 24-Methylenecholesterol, Delta7-Avenasterol, 5-Dehydroavenasterol, 4alpha-Methylzymosterol-4-carboxylate, 3-Keto-4-methylzymosterol, 4,4-Dimethyl-5alpha-cholesta-8-en-3beta-ol, Calcitetrol, 24-epi-Campesterol, 4alpha-Carboxy-5alpha-cholesta-8,24-dien-3beta-ol, 3beta-Hydroxy-4beta,14alpha-dimethyl-9beta,19-cyclo-5alpha-ergost-24(24(1))-en-4alpha-carboxylate, 3beta-Hydroxyergosta-7,24(24(1))-dien-4alpha-carboxylate, 4alpha-Carboxy-stigmasta-7,24(24(1))-dien-3beta-ol, Cycloeucalenone, Episterone, Avenastenone, Zymosterone
97 M00104_C00695 hsa00120 Primary bile acid biosynthesis Primary bile acid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Bile acids are steroid carboxylic acids derived from cholesterol in vertebrates. The primary bile acids, cholic acid and chenodeoxycholic acid, are synthesized in the liver and conjugated with taurine or glycine before secretion via bile into the intestine. The conversion from cholesterol to cholic and chenodeoxycholic acids involves four steps: 1) the initiation of synthesis by 7alpha-hydroxylation of sterol precursors, 2) further modifications to the ring structures, 3) side-chain oxidation and shortening (cleavage) by three carbons, and 4) conjugation of the bile acid with taurine or glycine. Glycine, Cholesterol, Taurine, Cholic acid, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-al, Choloyl-CoA, Glycocholate, Chenodeoxycholate, 7alpha-Hydroxycholesterol, 3alpha,7alpha-Dihydroxy-5beta-cholestanate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoate, Taurocholate, Chenodeoxycholoyl-CoA, 3alpha,7alpha,26-Trihydroxy-5beta-cholestane, 3alpha,7alpha-Dihydroxy-5beta-cholestan-26-al, 3alpha,7alpha,12alpha,26-Tetrahydroxy-5beta-cholestane, (24E)-3alpha,7alpha-Dihydroxy-5beta-cholest-24-enoyl-CoA, (24R,25R)-3alpha,7alpha,24-Trihydroxy-5beta-cholestanoyl-CoA, 3alpha,7alpha-Dihydroxy-5beta-24-oxocholestanoyl-CoA, 7alpha-Hydroxy-5beta-cholestan-3-one, 3alpha,7alpha-Dihydroxy-5beta-cholestane, 7alpha,12alpha-Dihydroxy-5beta-cholestan-3-one, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestane, 7alpha-Hydroxycholest-4-en-3-one, (24E)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholest-24-enoyl-CoA, Taurochenodeoxycholate, Glycochenodeoxycholate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-24-oxocholestanoyl-CoA, 5beta-Cyprinolsulfate, 7alpha,27-Dihydroxycholesterol, Cerebrosterol, (24S)-Cholest-5-ene-3beta,7alpha,24-triol, 25-Hydroxycholesterol, Cholest-5-ene-3beta,7alpha,25-triol, Cholest-5-ene-3beta,26-diol, (25R)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (24R,25R)-3alpha,7alpha,12alpha,24-Tetrahydroxy-5beta-cholestanoyl-CoA, 7alpha,24-Dihydroxy-4-cholesten-3-one, 7alpha,25-Dihydroxy-4-cholesten-3-one, 3beta-Hydroxy-5-cholestenoate, 3beta,7alpha-Dihydroxy-5-cholestenoate, 7alpha,26-Dihydroxy-4-cholesten-3-one, 7alpha-Hydroxy-3-oxo-4-cholestenoate, 4-Cholesten-7alpha,12alpha-diol-3-one, (25S)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (25R)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA, (25S)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA
98 M00104_C02528 hsa00120 Primary bile acid biosynthesis Primary bile acid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Bile acids are steroid carboxylic acids derived from cholesterol in vertebrates. The primary bile acids, cholic acid and chenodeoxycholic acid, are synthesized in the liver and conjugated with taurine or glycine before secretion via bile into the intestine. The conversion from cholesterol to cholic and chenodeoxycholic acids involves four steps: 1) the initiation of synthesis by 7alpha-hydroxylation of sterol precursors, 2) further modifications to the ring structures, 3) side-chain oxidation and shortening (cleavage) by three carbons, and 4) conjugation of the bile acid with taurine or glycine. Glycine, Cholesterol, Taurine, Cholic acid, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-al, Choloyl-CoA, Glycocholate, Chenodeoxycholate, 7alpha-Hydroxycholesterol, 3alpha,7alpha-Dihydroxy-5beta-cholestanate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoate, Taurocholate, Chenodeoxycholoyl-CoA, 3alpha,7alpha,26-Trihydroxy-5beta-cholestane, 3alpha,7alpha-Dihydroxy-5beta-cholestan-26-al, 3alpha,7alpha,12alpha,26-Tetrahydroxy-5beta-cholestane, (24E)-3alpha,7alpha-Dihydroxy-5beta-cholest-24-enoyl-CoA, (24R,25R)-3alpha,7alpha,24-Trihydroxy-5beta-cholestanoyl-CoA, 3alpha,7alpha-Dihydroxy-5beta-24-oxocholestanoyl-CoA, 7alpha-Hydroxy-5beta-cholestan-3-one, 3alpha,7alpha-Dihydroxy-5beta-cholestane, 7alpha,12alpha-Dihydroxy-5beta-cholestan-3-one, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestane, 7alpha-Hydroxycholest-4-en-3-one, (24E)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholest-24-enoyl-CoA, Taurochenodeoxycholate, Glycochenodeoxycholate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-24-oxocholestanoyl-CoA, 5beta-Cyprinolsulfate, 7alpha,27-Dihydroxycholesterol, Cerebrosterol, (24S)-Cholest-5-ene-3beta,7alpha,24-triol, 25-Hydroxycholesterol, Cholest-5-ene-3beta,7alpha,25-triol, Cholest-5-ene-3beta,26-diol, (25R)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (24R,25R)-3alpha,7alpha,12alpha,24-Tetrahydroxy-5beta-cholestanoyl-CoA, 7alpha,24-Dihydroxy-4-cholesten-3-one, 7alpha,25-Dihydroxy-4-cholesten-3-one, 3beta-Hydroxy-5-cholestenoate, 3beta,7alpha-Dihydroxy-5-cholestenoate, 7alpha,26-Dihydroxy-4-cholesten-3-one, 7alpha-Hydroxy-3-oxo-4-cholestenoate, 4-Cholesten-7alpha,12alpha-diol-3-one, (25S)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (25R)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA, (25S)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA
99 M00106_C05122 hsa00120 Primary bile acid biosynthesis Primary bile acid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Bile acids are steroid carboxylic acids derived from cholesterol in vertebrates. The primary bile acids, cholic acid and chenodeoxycholic acid, are synthesized in the liver and conjugated with taurine or glycine before secretion via bile into the intestine. The conversion from cholesterol to cholic and chenodeoxycholic acids involves four steps: 1) the initiation of synthesis by 7alpha-hydroxylation of sterol precursors, 2) further modifications to the ring structures, 3) side-chain oxidation and shortening (cleavage) by three carbons, and 4) conjugation of the bile acid with taurine or glycine. Glycine, Cholesterol, Taurine, Cholic acid, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-al, Choloyl-CoA, Glycocholate, Chenodeoxycholate, 7alpha-Hydroxycholesterol, 3alpha,7alpha-Dihydroxy-5beta-cholestanate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoate, Taurocholate, Chenodeoxycholoyl-CoA, 3alpha,7alpha,26-Trihydroxy-5beta-cholestane, 3alpha,7alpha-Dihydroxy-5beta-cholestan-26-al, 3alpha,7alpha,12alpha,26-Tetrahydroxy-5beta-cholestane, (24E)-3alpha,7alpha-Dihydroxy-5beta-cholest-24-enoyl-CoA, (24R,25R)-3alpha,7alpha,24-Trihydroxy-5beta-cholestanoyl-CoA, 3alpha,7alpha-Dihydroxy-5beta-24-oxocholestanoyl-CoA, 7alpha-Hydroxy-5beta-cholestan-3-one, 3alpha,7alpha-Dihydroxy-5beta-cholestane, 7alpha,12alpha-Dihydroxy-5beta-cholestan-3-one, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestane, 7alpha-Hydroxycholest-4-en-3-one, (24E)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholest-24-enoyl-CoA, Taurochenodeoxycholate, Glycochenodeoxycholate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-24-oxocholestanoyl-CoA, 5beta-Cyprinolsulfate, 7alpha,27-Dihydroxycholesterol, Cerebrosterol, (24S)-Cholest-5-ene-3beta,7alpha,24-triol, 25-Hydroxycholesterol, Cholest-5-ene-3beta,7alpha,25-triol, Cholest-5-ene-3beta,26-diol, (25R)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (24R,25R)-3alpha,7alpha,12alpha,24-Tetrahydroxy-5beta-cholestanoyl-CoA, 7alpha,24-Dihydroxy-4-cholesten-3-one, 7alpha,25-Dihydroxy-4-cholesten-3-one, 3beta-Hydroxy-5-cholestenoate, 3beta,7alpha-Dihydroxy-5-cholestenoate, 7alpha,26-Dihydroxy-4-cholesten-3-one, 7alpha-Hydroxy-3-oxo-4-cholestenoate, 4-Cholesten-7alpha,12alpha-diol-3-one, (25S)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (25R)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA, (25S)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA
100 M00106_C01921 hsa00120 Primary bile acid biosynthesis Primary bile acid biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Bile acids are steroid carboxylic acids derived from cholesterol in vertebrates. The primary bile acids, cholic acid and chenodeoxycholic acid, are synthesized in the liver and conjugated with taurine or glycine before secretion via bile into the intestine. The conversion from cholesterol to cholic and chenodeoxycholic acids involves four steps: 1) the initiation of synthesis by 7alpha-hydroxylation of sterol precursors, 2) further modifications to the ring structures, 3) side-chain oxidation and shortening (cleavage) by three carbons, and 4) conjugation of the bile acid with taurine or glycine. Glycine, Cholesterol, Taurine, Cholic acid, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-al, Choloyl-CoA, Glycocholate, Chenodeoxycholate, 7alpha-Hydroxycholesterol, 3alpha,7alpha-Dihydroxy-5beta-cholestanate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestanoate, Taurocholate, Chenodeoxycholoyl-CoA, 3alpha,7alpha,26-Trihydroxy-5beta-cholestane, 3alpha,7alpha-Dihydroxy-5beta-cholestan-26-al, 3alpha,7alpha,12alpha,26-Tetrahydroxy-5beta-cholestane, (24E)-3alpha,7alpha-Dihydroxy-5beta-cholest-24-enoyl-CoA, (24R,25R)-3alpha,7alpha,24-Trihydroxy-5beta-cholestanoyl-CoA, 3alpha,7alpha-Dihydroxy-5beta-24-oxocholestanoyl-CoA, 7alpha-Hydroxy-5beta-cholestan-3-one, 3alpha,7alpha-Dihydroxy-5beta-cholestane, 7alpha,12alpha-Dihydroxy-5beta-cholestan-3-one, 3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestane, 7alpha-Hydroxycholest-4-en-3-one, (24E)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholest-24-enoyl-CoA, Taurochenodeoxycholate, Glycochenodeoxycholate, 3alpha,7alpha,12alpha-Trihydroxy-5beta-24-oxocholestanoyl-CoA, 5beta-Cyprinolsulfate, 7alpha,27-Dihydroxycholesterol, Cerebrosterol, (24S)-Cholest-5-ene-3beta,7alpha,24-triol, 25-Hydroxycholesterol, Cholest-5-ene-3beta,7alpha,25-triol, Cholest-5-ene-3beta,26-diol, (25R)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (24R,25R)-3alpha,7alpha,12alpha,24-Tetrahydroxy-5beta-cholestanoyl-CoA, 7alpha,24-Dihydroxy-4-cholesten-3-one, 7alpha,25-Dihydroxy-4-cholesten-3-one, 3beta-Hydroxy-5-cholestenoate, 3beta,7alpha-Dihydroxy-5-cholestenoate, 7alpha,26-Dihydroxy-4-cholesten-3-one, 7alpha-Hydroxy-3-oxo-4-cholestenoate, 4-Cholesten-7alpha,12alpha-diol-3-one, (25S)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholestan-26-oyl-CoA, (25R)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA, (25S)-3alpha,7alpha-Dihydroxy-5beta-cholestanoyl-CoA
101 M00107_C00410 hsa00140 Steroid hormone biosynthesis Steroid hormone biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Steroid hormones derived from cholesterol are a class of biologically active compounds in vertebrates. The cholesterol side-chain cleavage enzyme CYP11A1 catalyzes conversion of cholesterol, a C27 compound, to the first C21 steroid, pregnenolone, which is converted by a bifunctional enzyme complex to the gestagen hormone, progesterone [MD:M00107]. Pregnenolone and progesterone are the starting materials for the three groups of steroids: C21 steroids of glucocorticoids and mineralocorticoids, C19 steroids of androgens, and C18 steroids of estrogens. (i) Progesterone is converted by hydroxylations at carbons 21 and 11 to corticosterone, which is further modified by hydroxylation and oxydoreduction at carbon 18 to yield aldosterone, a mineralcorticoid [MD:M00108]. Cortisol, the main glucocorticoid, is formed from 17alpha-hydroxyprogesterone with 11-deoxycortisol as an intermediate [MD:M00109]. (ii) Male hormone testosterone is formed from pregnenolone by two pathways, delta5 pathway via dehydroepiandrosterone and delta4 pathway via androstenedione [MD:M00110]. The enzyme CYP17A1 is responsible for the 17,20 lyase and 17alpha-hydroxylase activities in respective pathways. (iii) Female hormones estrone and estradiol are formed from testosterone and 4-androstene-3,17-dione by oxidative removal of the C19 methyl group and subsequent aromatization of ring A. In addition to these three groups, recent studies show that there is another group, termed neurosteroids, synthesized in the brain rather than the peripheral endocrine gland. Cholesterol, Androstenedione, Progesterone, Estrone, Androsterone, Testosterone, 5alpha-Androstane-3,17-dione, Cortisol, Cortisone, Estradiol-17beta, 18-Hydroxycorticosterone, 17alpha-Hydroxyprogesterone, Dehydroepiandrosterone, Aldosterone, Pregnenolone, Corticosterone, 4-Methylpentanal, Estradiol-17alpha, Estrone 3-sulfate, 11-Deoxycorticosterone, 5alpha-Pregnane-3,20-dione, 11alpha-Hydroxyprogesterone, 5beta-Androstane-3,17-dione, Androstan-3alpha,17beta-diol, Dihydrotestosterone, 6beta-Hydroxy-17beta-estradiol, 20alpha-Hydroxy-4-pregnen-3-one, Androstenediol, Etiocholanolone, 17alpha,20alpha-Dihydroxypregn-4-en-3-one, Dehydroepiandrosterone sulfate, Testololactone, 17alpha-Hydroxypregnenolone, 16alpha-Hydroxydehydroepiandrosterone, 16alpha-Hydroxyandrost-4-ene-3,17-dione, Estriol, 11beta-Hydroxyandrost-4-ene-3,17-dione, Adrenosterone, 19-Hydroxyandrost-4-ene-3,17-dione, 7alpha-Hydroxytestosterone, 5beta-Dihydrotestosterone, 19-Hydroxytestosterone, 19-Oxotestosterone, 7alpha-Hydroxyandrost-4-ene-3,17-dione, 19-Oxoandrost-4-ene-3,17-dione, 2-Hydroxyestrone, 2-Methoxyestrone, 16alpha-Hydroxyestrone, 2-Hydroxyestradiol, 2-Methoxy-17beta-estradiol, 17alpha,21-Dihydroxy-5beta-pregnane-3,11,20-trione, Tetrahydrocortisone, Dihydrocortisol, Urocortisol, 11beta,21-Dihydroxy-3,20-oxo-5beta-pregnan-18-al, 3alpha,11beta,21-Trihydroxy-20-oxo-5beta-pregnan-18-al, 11beta,21-Dihydroxy-5beta-pregnane-3,20-dione, Tetrahydrocorticosterone, 21-Hydroxy-5beta-pregnane-3,11,20-trione, 3alpha,21-Dihydroxy-5beta-pregnane-11,20-dione, 5beta-Pregnane-3,20-dione, Pregnanolone, Cortolone, Cortol, 3alpha,20alpha,21-Trihydroxy-5beta-pregnan-11-one, Pregnanediol, 21-Hydroxypregnenolone, 17alpha,21-Dihydroxypregnenolone, 11-Deoxycortisol, 11beta,17alpha,21-Trihydroxypregnenolone, 11-Dehydrocorticosterone, 21-Deoxycortisol, 11beta-Hydroxyprogesterone, 17alpha,20alpha-Dihydroxycholesterol, 20alpha-Hydroxycholesterol, 20alpha,22beta-Dihydroxycholesterol, 22(R)-Hydroxycholesterol, Estradiol-17beta 3-glucuronide, 16-Glucuronide-estriol, Estradiol-17beta 3-sulfate, 2-Methoxyestrone 3-sulfate, 2-Methoxyestradiol-17beta 3-sulfate, 2-Methoxy-estradiol-17beta 3-glucuronide, 2-Methoxyestrone 3-glucuronide, Estrone glucuronide, Testosterone glucuronide, Androsterone glucuronide, Etiocholan-3alpha-ol-17-one 3-glucuronide, Allopregnanolone, Allotetrahydrodeoxycorticosterone, 7alpha-Hydroxypregnenolone, Aldosterone hemiacetal, 5alpha-Dihydrodeoxycorticosterone, 5alpha-Pregnan-20alpha-ol-3-one, 5alpha-Pregnane-3alpha,20alpha-diol, Cholesterol sulfate, 3beta-Hydroxypregn-5-en-20-one sulfate, 7alpha-Hydroxydehydroepiandrosterone, 11beta,17beta-Dihydroxy-4-androsten-3-one, 5alpha-Pregnan-17alpha-ol-3,20-dione, 5alpha-Pregnane-3alpha,17alpha-diol-20-one
102 M00108_C01780 hsa00140 Steroid hormone biosynthesis Steroid hormone biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Steroid hormones derived from cholesterol are a class of biologically active compounds in vertebrates. The cholesterol side-chain cleavage enzyme CYP11A1 catalyzes conversion of cholesterol, a C27 compound, to the first C21 steroid, pregnenolone, which is converted by a bifunctional enzyme complex to the gestagen hormone, progesterone [MD:M00107]. Pregnenolone and progesterone are the starting materials for the three groups of steroids: C21 steroids of glucocorticoids and mineralocorticoids, C19 steroids of androgens, and C18 steroids of estrogens. (i) Progesterone is converted by hydroxylations at carbons 21 and 11 to corticosterone, which is further modified by hydroxylation and oxydoreduction at carbon 18 to yield aldosterone, a mineralcorticoid [MD:M00108]. Cortisol, the main glucocorticoid, is formed from 17alpha-hydroxyprogesterone with 11-deoxycortisol as an intermediate [MD:M00109]. (ii) Male hormone testosterone is formed from pregnenolone by two pathways, delta5 pathway via dehydroepiandrosterone and delta4 pathway via androstenedione [MD:M00110]. The enzyme CYP17A1 is responsible for the 17,20 lyase and 17alpha-hydroxylase activities in respective pathways. (iii) Female hormones estrone and estradiol are formed from testosterone and 4-androstene-3,17-dione by oxidative removal of the C19 methyl group and subsequent aromatization of ring A. In addition to these three groups, recent studies show that there is another group, termed neurosteroids, synthesized in the brain rather than the peripheral endocrine gland. Cholesterol, Androstenedione, Progesterone, Estrone, Androsterone, Testosterone, 5alpha-Androstane-3,17-dione, Cortisol, Cortisone, Estradiol-17beta, 18-Hydroxycorticosterone, 17alpha-Hydroxyprogesterone, Dehydroepiandrosterone, Aldosterone, Pregnenolone, Corticosterone, 4-Methylpentanal, Estradiol-17alpha, Estrone 3-sulfate, 11-Deoxycorticosterone, 5alpha-Pregnane-3,20-dione, 11alpha-Hydroxyprogesterone, 5beta-Androstane-3,17-dione, Androstan-3alpha,17beta-diol, Dihydrotestosterone, 6beta-Hydroxy-17beta-estradiol, 20alpha-Hydroxy-4-pregnen-3-one, Androstenediol, Etiocholanolone, 17alpha,20alpha-Dihydroxypregn-4-en-3-one, Dehydroepiandrosterone sulfate, Testololactone, 17alpha-Hydroxypregnenolone, 16alpha-Hydroxydehydroepiandrosterone, 16alpha-Hydroxyandrost-4-ene-3,17-dione, Estriol, 11beta-Hydroxyandrost-4-ene-3,17-dione, Adrenosterone, 19-Hydroxyandrost-4-ene-3,17-dione, 7alpha-Hydroxytestosterone, 5beta-Dihydrotestosterone, 19-Hydroxytestosterone, 19-Oxotestosterone, 7alpha-Hydroxyandrost-4-ene-3,17-dione, 19-Oxoandrost-4-ene-3,17-dione, 2-Hydroxyestrone, 2-Methoxyestrone, 16alpha-Hydroxyestrone, 2-Hydroxyestradiol, 2-Methoxy-17beta-estradiol, 17alpha,21-Dihydroxy-5beta-pregnane-3,11,20-trione, Tetrahydrocortisone, Dihydrocortisol, Urocortisol, 11beta,21-Dihydroxy-3,20-oxo-5beta-pregnan-18-al, 3alpha,11beta,21-Trihydroxy-20-oxo-5beta-pregnan-18-al, 11beta,21-Dihydroxy-5beta-pregnane-3,20-dione, Tetrahydrocorticosterone, 21-Hydroxy-5beta-pregnane-3,11,20-trione, 3alpha,21-Dihydroxy-5beta-pregnane-11,20-dione, 5beta-Pregnane-3,20-dione, Pregnanolone, Cortolone, Cortol, 3alpha,20alpha,21-Trihydroxy-5beta-pregnan-11-one, Pregnanediol, 21-Hydroxypregnenolone, 17alpha,21-Dihydroxypregnenolone, 11-Deoxycortisol, 11beta,17alpha,21-Trihydroxypregnenolone, 11-Dehydrocorticosterone, 21-Deoxycortisol, 11beta-Hydroxyprogesterone, 17alpha,20alpha-Dihydroxycholesterol, 20alpha-Hydroxycholesterol, 20alpha,22beta-Dihydroxycholesterol, 22(R)-Hydroxycholesterol, Estradiol-17beta 3-glucuronide, 16-Glucuronide-estriol, Estradiol-17beta 3-sulfate, 2-Methoxyestrone 3-sulfate, 2-Methoxyestradiol-17beta 3-sulfate, 2-Methoxy-estradiol-17beta 3-glucuronide, 2-Methoxyestrone 3-glucuronide, Estrone glucuronide, Testosterone glucuronide, Androsterone glucuronide, Etiocholan-3alpha-ol-17-one 3-glucuronide, Allopregnanolone, Allotetrahydrodeoxycorticosterone, 7alpha-Hydroxypregnenolone, Aldosterone hemiacetal, 5alpha-Dihydrodeoxycorticosterone, 5alpha-Pregnan-20alpha-ol-3-one, 5alpha-Pregnane-3alpha,20alpha-diol, Cholesterol sulfate, 3beta-Hydroxypregn-5-en-20-one sulfate, 7alpha-Hydroxydehydroepiandrosterone, 11beta,17beta-Dihydroxy-4-androsten-3-one, 5alpha-Pregnan-17alpha-ol-3,20-dione, 5alpha-Pregnane-3alpha,17alpha-diol-20-one
103 M00109_C00762 hsa00140 Steroid hormone biosynthesis Steroid hormone biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Steroid hormones derived from cholesterol are a class of biologically active compounds in vertebrates. The cholesterol side-chain cleavage enzyme CYP11A1 catalyzes conversion of cholesterol, a C27 compound, to the first C21 steroid, pregnenolone, which is converted by a bifunctional enzyme complex to the gestagen hormone, progesterone [MD:M00107]. Pregnenolone and progesterone are the starting materials for the three groups of steroids: C21 steroids of glucocorticoids and mineralocorticoids, C19 steroids of androgens, and C18 steroids of estrogens. (i) Progesterone is converted by hydroxylations at carbons 21 and 11 to corticosterone, which is further modified by hydroxylation and oxydoreduction at carbon 18 to yield aldosterone, a mineralcorticoid [MD:M00108]. Cortisol, the main glucocorticoid, is formed from 17alpha-hydroxyprogesterone with 11-deoxycortisol as an intermediate [MD:M00109]. (ii) Male hormone testosterone is formed from pregnenolone by two pathways, delta5 pathway via dehydroepiandrosterone and delta4 pathway via androstenedione [MD:M00110]. The enzyme CYP17A1 is responsible for the 17,20 lyase and 17alpha-hydroxylase activities in respective pathways. (iii) Female hormones estrone and estradiol are formed from testosterone and 4-androstene-3,17-dione by oxidative removal of the C19 methyl group and subsequent aromatization of ring A. In addition to these three groups, recent studies show that there is another group, termed neurosteroids, synthesized in the brain rather than the peripheral endocrine gland. Cholesterol, Androstenedione, Progesterone, Estrone, Androsterone, Testosterone, 5alpha-Androstane-3,17-dione, Cortisol, Cortisone, Estradiol-17beta, 18-Hydroxycorticosterone, 17alpha-Hydroxyprogesterone, Dehydroepiandrosterone, Aldosterone, Pregnenolone, Corticosterone, 4-Methylpentanal, Estradiol-17alpha, Estrone 3-sulfate, 11-Deoxycorticosterone, 5alpha-Pregnane-3,20-dione, 11alpha-Hydroxyprogesterone, 5beta-Androstane-3,17-dione, Androstan-3alpha,17beta-diol, Dihydrotestosterone, 6beta-Hydroxy-17beta-estradiol, 20alpha-Hydroxy-4-pregnen-3-one, Androstenediol, Etiocholanolone, 17alpha,20alpha-Dihydroxypregn-4-en-3-one, Dehydroepiandrosterone sulfate, Testololactone, 17alpha-Hydroxypregnenolone, 16alpha-Hydroxydehydroepiandrosterone, 16alpha-Hydroxyandrost-4-ene-3,17-dione, Estriol, 11beta-Hydroxyandrost-4-ene-3,17-dione, Adrenosterone, 19-Hydroxyandrost-4-ene-3,17-dione, 7alpha-Hydroxytestosterone, 5beta-Dihydrotestosterone, 19-Hydroxytestosterone, 19-Oxotestosterone, 7alpha-Hydroxyandrost-4-ene-3,17-dione, 19-Oxoandrost-4-ene-3,17-dione, 2-Hydroxyestrone, 2-Methoxyestrone, 16alpha-Hydroxyestrone, 2-Hydroxyestradiol, 2-Methoxy-17beta-estradiol, 17alpha,21-Dihydroxy-5beta-pregnane-3,11,20-trione, Tetrahydrocortisone, Dihydrocortisol, Urocortisol, 11beta,21-Dihydroxy-3,20-oxo-5beta-pregnan-18-al, 3alpha,11beta,21-Trihydroxy-20-oxo-5beta-pregnan-18-al, 11beta,21-Dihydroxy-5beta-pregnane-3,20-dione, Tetrahydrocorticosterone, 21-Hydroxy-5beta-pregnane-3,11,20-trione, 3alpha,21-Dihydroxy-5beta-pregnane-11,20-dione, 5beta-Pregnane-3,20-dione, Pregnanolone, Cortolone, Cortol, 3alpha,20alpha,21-Trihydroxy-5beta-pregnan-11-one, Pregnanediol, 21-Hydroxypregnenolone, 17alpha,21-Dihydroxypregnenolone, 11-Deoxycortisol, 11beta,17alpha,21-Trihydroxypregnenolone, 11-Dehydrocorticosterone, 21-Deoxycortisol, 11beta-Hydroxyprogesterone, 17alpha,20alpha-Dihydroxycholesterol, 20alpha-Hydroxycholesterol, 20alpha,22beta-Dihydroxycholesterol, 22(R)-Hydroxycholesterol, Estradiol-17beta 3-glucuronide, 16-Glucuronide-estriol, Estradiol-17beta 3-sulfate, 2-Methoxyestrone 3-sulfate, 2-Methoxyestradiol-17beta 3-sulfate, 2-Methoxy-estradiol-17beta 3-glucuronide, 2-Methoxyestrone 3-glucuronide, Estrone glucuronide, Testosterone glucuronide, Androsterone glucuronide, Etiocholan-3alpha-ol-17-one 3-glucuronide, Allopregnanolone, Allotetrahydrodeoxycorticosterone, 7alpha-Hydroxypregnenolone, Aldosterone hemiacetal, 5alpha-Dihydrodeoxycorticosterone, 5alpha-Pregnan-20alpha-ol-3-one, 5alpha-Pregnane-3alpha,20alpha-diol, Cholesterol sulfate, 3beta-Hydroxypregn-5-en-20-one sulfate, 7alpha-Hydroxydehydroepiandrosterone, 11beta,17beta-Dihydroxy-4-androsten-3-one, 5alpha-Pregnan-17alpha-ol-3,20-dione, 5alpha-Pregnane-3alpha,17alpha-diol-20-one
104 M00110_C00468 hsa00140 Steroid hormone biosynthesisf Steroid hormone biosynthesis - Homo sapiens (human) Metabolism; Lipid metabolism Steroid hormones derived from cholesterol are a class of biologically active compounds in vertebrates. The cholesterol side-chain cleavage enzyme CYP11A1 catalyzes conversion of cholesterol, a C27 compound, to the first C21 steroid, pregnenolone, which is converted by a bifunctional enzyme complex to the gestagen hormone, progesterone [MD:M00107]. Pregnenolone and progesterone are the starting materials for the three groups of steroids: C21 steroids of glucocorticoids and mineralocorticoids, C19 steroids of androgens, and C18 steroids of estrogens. (i) Progesterone is converted by hydroxylations at carbons 21 and 11 to corticosterone, which is further modified by hydroxylation and oxydoreduction at carbon 18 to yield aldosterone, a mineralcorticoid [MD:M00108]. Cortisol, the main glucocorticoid, is formed from 17alpha-hydroxyprogesterone with 11-deoxycortisol as an intermediate [MD:M00109]. (ii) Male hormone testosterone is formed from pregnenolone by two pathways, delta5 pathway via dehydroepiandrosterone and delta4 pathway via androstenedione [MD:M00110]. The enzyme CYP17A1 is responsible for the 17,20 lyase and 17alpha-hydroxylase activities in respective pathways. (iii) Female hormones estrone and estradiol are formed from testosterone and 4-androstene-3,17-dione by oxidative removal of the C19 methyl group and subsequent aromatization of ring A. In addition to these three groups, recent studies show that there is another group, termed neurosteroids, synthesized in the brain rather than the peripheral endocrine gland. Cholesterol, Androstenedione, Progesterone, Estrone, Androsterone, Testosterone, 5alpha-Androstane-3,17-dione, Cortisol, Cortisone, Estradiol-17beta, 18-Hydroxycorticosterone, 17alpha-Hydroxyprogesterone, Dehydroepiandrosterone, Aldosterone, Pregnenolone, Corticosterone, 4-Methylpentanal, Estradiol-17alpha, Estrone 3-sulfate, 11-Deoxycorticosterone, 5alpha-Pregnane-3,20-dione, 11alpha-Hydroxyprogesterone, 5beta-Androstane-3,17-dione, Androstan-3alpha,17beta-diol, Dihydrotestosterone, 6beta-Hydroxy-17beta-estradiol, 20alpha-Hydroxy-4-pregnen-3-one, Androstenediol, Etiocholanolone, 17alpha,20alpha-Dihydroxypregn-4-en-3-one, Dehydroepiandrosterone sulfate, Testololactone, 17alpha-Hydroxypregnenolone, 16alpha-Hydroxydehydroepiandrosterone, 16alpha-Hydroxyandrost-4-ene-3,17-dione, Estriol, 11beta-Hydroxyandrost-4-ene-3,17-dione, Adrenosterone, 19-Hydroxyandrost-4-ene-3,17-dione, 7alpha-Hydroxytestosterone, 5beta-Dihydrotestosterone, 19-Hydroxytestosterone, 19-Oxotestosterone, 7alpha-Hydroxyandrost-4-ene-3,17-dione, 19-Oxoandrost-4-ene-3,17-dione, 2-Hydroxyestrone, 2-Methoxyestrone, 16alpha-Hydroxyestrone, 2-Hydroxyestradiol, 2-Methoxy-17beta-estradiol, 17alpha,21-Dihydroxy-5beta-pregnane-3,11,20-trione, Tetrahydrocortisone, Dihydrocortisol, Urocortisol, 11beta,21-Dihydroxy-3,20-oxo-5beta-pregnan-18-al, 3alpha,11beta,21-Trihydroxy-20-oxo-5beta-pregnan-18-al, 11beta,21-Dihydroxy-5beta-pregnane-3,20-dione, Tetrahydrocorticosterone, 21-Hydroxy-5beta-pregnane-3,11,20-trione, 3alpha,21-Dihydroxy-5beta-pregnane-11,20-dione, 5beta-Pregnane-3,20-dione, Pregnanolone, Cortolone, Cortol, 3alpha,20alpha,21-Trihydroxy-5beta-pregnan-11-one, Pregnanediol, 21-Hydroxypregnenolone, 17alpha,21-Dihydroxypregnenolone, 11-Deoxycortisol, 11beta,17alpha,21-Trihydroxypregnenolone, 11-Dehydrocorticosterone, 21-Deoxycortisol, 11beta-Hydroxyprogesterone, 17alpha,20alpha-Dihydroxycholesterol, 20alpha-Hydroxycholesterol, 20alpha,22beta-Dihydroxycholesterol, 22(R)-Hydroxycholesterol, Estradiol-17beta 3-glucuronide, 16-Glucuronide-estriol, Estradiol-17beta 3-sulfate, 2-Methoxyestrone 3-sulfate, 2-Methoxyestradiol-17beta 3-sulfate, 2-Methoxy-estradiol-17beta 3-glucuronide, 2-Methoxyestrone 3-glucuronide, Estrone glucuronide, Testosterone glucuronide, Androsterone glucuronide, Etiocholan-3alpha-ol-17-one 3-glucuronide, Allopregnanolone, Allotetrahydrodeoxycorticosterone, 7alpha-Hydroxypregnenolone, Aldosterone hemiacetal, 5alpha-Dihydrodeoxycorticosterone, 5alpha-Pregnan-20alpha-ol-3-one, 5alpha-Pregnane-3alpha,20alpha-diol, Cholesterol sulfate, 3beta-Hydroxypregn-5-en-20-one sulfate, 7alpha-Hydroxydehydroepiandrosterone, 11beta,17beta-Dihydroxy-4-androsten-3-one, 5alpha-Pregnan-17alpha-ol-3,20-dione, 5alpha-Pregnane-3alpha,17alpha-diol-20-one
105 M00118_C00051 hsa00480 Glutathione metabolism Glutathione metabolism - Homo sapiens (human) Metabolism; Metabolism of other amino acids NA NADPH, NADP+, Acetyl-CoA, L-Glutamate, Glycine, Glutathione, Ascorbate, L-Ornithine, L-Cysteine, Glutathione disulfide, Putrescine, L-Amino acid, Spermidine, gamma-L-Glutamyl-L-cysteine, Spermine, RX, Cys-Gly, Cadaverine, 5-Oxoproline, Trypanothione, R-S-Glutathione, Trypanothione disulfide, Bis-gamma-glutamylcystine, (5-L-Glutamyl)-L-amino acid, Dehydroascorbate, S-Substituted L-cysteine, S-Substituted N-acetyl-L-cysteine, R-S-Cysteinylglycine, Glutathionylspermidine, Glutathionylspermine, Bis(glutathionyl)spermine, Bis(glutathionyl)spermine disulfide, Aminopropylcadaverine, Glutathionylaminopropylcadaverine, Homotrypanothione, Homotrypanothione disulfide, Tryparedoxin, Tryparedoxin disulfide
106 M00120_C00010 hsa00770 Pantothenate and CoA biosynthesis Pantothenate and CoA biosynthesis - Homo sapiens (human) Metabolism; Metabolism of cofactors and vitamins NA CoA, Pyruvate, L-Aspartate, Adenosine 3',5'-bisphosphate, L-Cysteine, beta-Alanine, Uracil, 3-Methyl-2-oxobutanoic acid, L-Valine, Acyl-carrier protein, 5,6-Dihydrouracil, (R)-Pantoate, Spermine, Pantetheine, Pantothenate, Dephospho-CoA, 2-Dehydropantoate, (R)-4-Dehydropantoate, (R)-3,3-Dimethylmalate, Pantetheine 4'-phosphate, 3-Ureidopropionate, D-4'-Phosphopantothenate, Apo-[acyl-carrier protein], N-((R)-Pantothenoyl)-L-cysteine, (R)-2,3-Dihydroxy-3-methylbutanoate, (R)-4'-Phosphopantothenoyl-L-cysteine, 3-Aminopropanal, Pantothenol, (S)-2-Acetolactate, (R)-4-Phosphopantoate
107 M00128_C00399 hsa00130 Ubiquinone and other terpenoid-quinone biosynthesis Ubiquinone and other terpenoid-quinone biosynthesis - Homo sapiens (human) Metabolism; Metabolism of cofactors and vitamins Ubiquinone (UQ), also called coenzyme Q, and plastoquinone (PQ) are electron carriers in oxidative phosphorylation and photosynthesis, respectively. The quinoid nucleus of ubiquinone is derived from the shikimate pathway; 4-hydroxybenzoate is directly formed from chorismate in bacteria, while it can be formed from either chorismate or tyrosine in yeast. The following biosynthesis of terpenoid moiety involves reactions of prenylation, decarboxylation, and three hydroxylations alternating with three methylations. The order of these reactions are somewhat different between bacteria and yeast. Phylloquinone (vitamin K1), menaquinone (vitamin K2), and tocopherol (vitamin E) are fat-soluble vitamins. Phylloquinone is a compound present in all photosynthetic plants serving as a cofactor for photosystem I-mediated electron transport. Menaquinone is an obligatory component of the electron-transfer pathway in bacteria. L-Tyrosine, 4-Hydroxybenzoate, p-Coumaroyl-CoA, Chorismate, Geranyl diphosphate, Geranylgeranyl diphosphate, Ubiquinol, trans-Cinnamate, Homogentisate, 4-Coumarate, Menaquinone, Isochorismate, 3-(4-Hydroxyphenyl)pyruvate, Phylloquinone, alpha-Tocopherol, gamma-Tocopherol, 2-Succinylbenzoate, 4-Hydroxybenzoyl-CoA, 2-Succinylbenzoyl-CoA, Phylloquinol, 1,4-Dihydroxy-2-naphthoate, (R)-3-(4-Hydroxyphenyl)lactate, 4-(beta-D-Glucosyloxy)benzoate, all-trans-Nonaprenyl diphosphate, Phytyl diphosphate, 2-Polyprenylphenol, (1R,6R)-6-Hydroxy-2-succinylcyclohexa-2,4-diene-1-carboxylate, Menaquinol, all-trans-Polyprenyl diphosphate, 4-Hydroxy-3-polyprenylbenzoate, Vitamin K1 epoxide, Spirodilactone, Geranylhydroquinone, 2-Phytyl-1,4-naphthoquinone, delta-Tocopherol, beta-Tocopherol, alpha-Tocotrienol, beta-Tocotrienol, gamma-Tocotrienol, delta-Tocotrienol, 1,4-Dihydroxy-2-naphthoyl-CoA, 2-Methyl-6-phytylquinol, 2,3-Dimethyl-5-phytylquinol, 2-Succinyl-5-enolpyruvyl-6-hydroxy-3-cyclohexene-1-carboxylate, Plastoquinol-9, Futalosine, Dehypoxanthine futalosine, Cyclic dehypoxanthine futalosine, 1,4-Dihydroxy-6-naphthoate, Shikonin, 2-Polyprenyl-6-hydroxyphenol, 2-Polyprenyl-6-methoxyphenol, 3,4-Dihydroxy-5-polyprenylbenzoate, 3-Methoxy-4-hydroxy-5-polyprenylbenzoate, 2-Methyl-6-solanyl-1,4-benzoquinol, Geranyl-hydroxybenzoate, 3''-Hydroxy-geranylhydroquinone, Deoxyshikonin, Dihydroechinofuran, Dihydroshikonofuran, Demethylmenaquinol, 2-Methoxy-6-all-trans-polyprenyl-1,4-benzoquinol, 6-Methoxy-3-methyl-2-all-trans-polyprenyl-1,4-benzoquinol, 2,3-Epoxymenaquinone, 6-Geranylgeranyl-2-methylbenzene-1,4-diol, 6-Geranylgeranyl-2,3-dimethylbenzene-1,4-diol, 3-[(1-Carboxyvinyl)oxy]benzoate, 6-Amino-6-deoxyfutalosine, Demethylphylloquinol, 3-Demethylubiquinol, 2-Carboxy-1,4-naphthoquinone
108 M00130_C01272 hsa00562 Inositol phosphate metabolism Inositol phosphate metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA Acetyl-CoA, D-Glucose 6-phosphate, Glycerone phosphate, D-Glyceraldehyde 3-phosphate, myo-Inositol, D-Glucuronate, 3-Oxopropanoate, D-Tagaturonate, 1,2-Diacyl-sn-glycerol, 2,4,6/3,5-Pentahydroxycyclohexanone, Inositol 1-phosphate, 1-Phosphatidyl-D-myo-inositol, Phytic acid, 1D-myo-Inositol 1,4-bisphosphate, 1D-myo-Inositol 1,3,4-trisphosphate, D-myo-Inositol 1,4,5-trisphosphate, 1D-myo-Inositol 1,3,4,5-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 4-phosphate, 1D-myo-Inositol 1,3,4,5,6-pentakisphosphate, myo-Inositol 4-phosphate, D-Bornesitol, L-Bornesitol, 1D-myo-Inositol 3-phosphate, D-myo-Inositol 1,3-bisphosphate, D-myo-Inositol 3,4-bisphosphate, 3D-3,5/4-Trihydroxycyclohexane-1,2-dione, 1D-myo-Inositol 1,3,4,6-tetrakisphosphate, 1D-myo-Inositol 3,4,5,6-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 3-phosphate, D-myo-Inositol 1,2,4,5,6-pentakisphosphate, Inositol 1,2,3,5,6-pentakisphosphate, 1-Phosphatidyl-D-myo-inositol 4,5-bisphosphate, Phosphatidylinositol-3,4,5-trisphosphate, scyllo-Inositol, 2-Deoxy-5-keto-D-gluconic acid, 2-Deoxy-5-keto-D-gluconic acid 6-phosphate, 1-Phosphatidyl-1D-myo-inositol 3,4-bisphosphate, 1D-myo-Inositol 1,4,5,6-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 3,5-bisphosphate, 1-Phosphatidyl-1D-myo-inositol 5-phosphate, 5-Deoxy-D-glucuronate, CDP-1L-myo-inositol, Bis(1L-myo-inositol)-3,1'-phosphate 1-phosphate, 1D-chiro-Inositol, 1-Keto-D-chiro-inositol, 3-Dehydro-scyllo-inosose, 5-Dehydro-L-gluconate
109 M00131_C00137 hsa00562 Inositol phosphate metabolism Inositol phosphate metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA Acetyl-CoA, D-Glucose 6-phosphate, Glycerone phosphate, D-Glyceraldehyde 3-phosphate, myo-Inositol, D-Glucuronate, 3-Oxopropanoate, D-Tagaturonate, 1,2-Diacyl-sn-glycerol, 2,4,6/3,5-Pentahydroxycyclohexanone, Inositol 1-phosphate, 1-Phosphatidyl-D-myo-inositol, Phytic acid, 1D-myo-Inositol 1,4-bisphosphate, 1D-myo-Inositol 1,3,4-trisphosphate, D-myo-Inositol 1,4,5-trisphosphate, 1D-myo-Inositol 1,3,4,5-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 4-phosphate, 1D-myo-Inositol 1,3,4,5,6-pentakisphosphate, myo-Inositol 4-phosphate, D-Bornesitol, L-Bornesitol, 1D-myo-Inositol 3-phosphate, D-myo-Inositol 1,3-bisphosphate, D-myo-Inositol 3,4-bisphosphate, 3D-3,5/4-Trihydroxycyclohexane-1,2-dione, 1D-myo-Inositol 1,3,4,6-tetrakisphosphate, 1D-myo-Inositol 3,4,5,6-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 3-phosphate, D-myo-Inositol 1,2,4,5,6-pentakisphosphate, Inositol 1,2,3,5,6-pentakisphosphate, 1-Phosphatidyl-D-myo-inositol 4,5-bisphosphate, Phosphatidylinositol-3,4,5-trisphosphate, scyllo-Inositol, 2-Deoxy-5-keto-D-gluconic acid, 2-Deoxy-5-keto-D-gluconic acid 6-phosphate, 1-Phosphatidyl-1D-myo-inositol 3,4-bisphosphate, 1D-myo-Inositol 1,4,5,6-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 3,5-bisphosphate, 1-Phosphatidyl-1D-myo-inositol 5-phosphate, 5-Deoxy-D-glucuronate, CDP-1L-myo-inositol, Bis(1L-myo-inositol)-3,1'-phosphate 1-phosphate, 1D-chiro-Inositol, 1-Keto-D-chiro-inositol, 3-Dehydro-scyllo-inosose, 5-Dehydro-L-gluconate
110 M00132_C01204 hsa00562 Inositol phosphate metabolism Inositol phosphate metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA Acetyl-CoA, D-Glucose 6-phosphate, Glycerone phosphate, D-Glyceraldehyde 3-phosphate, myo-Inositol, D-Glucuronate, 3-Oxopropanoate, D-Tagaturonate, 1,2-Diacyl-sn-glycerol, 2,4,6/3,5-Pentahydroxycyclohexanone, Inositol 1-phosphate, 1-Phosphatidyl-D-myo-inositol, Phytic acid, 1D-myo-Inositol 1,4-bisphosphate, 1D-myo-Inositol 1,3,4-trisphosphate, D-myo-Inositol 1,4,5-trisphosphate, 1D-myo-Inositol 1,3,4,5-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 4-phosphate, 1D-myo-Inositol 1,3,4,5,6-pentakisphosphate, myo-Inositol 4-phosphate, D-Bornesitol, L-Bornesitol, 1D-myo-Inositol 3-phosphate, D-myo-Inositol 1,3-bisphosphate, D-myo-Inositol 3,4-bisphosphate, 3D-3,5/4-Trihydroxycyclohexane-1,2-dione, 1D-myo-Inositol 1,3,4,6-tetrakisphosphate, 1D-myo-Inositol 3,4,5,6-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 3-phosphate, D-myo-Inositol 1,2,4,5,6-pentakisphosphate, Inositol 1,2,3,5,6-pentakisphosphate, 1-Phosphatidyl-D-myo-inositol 4,5-bisphosphate, Phosphatidylinositol-3,4,5-trisphosphate, scyllo-Inositol, 2-Deoxy-5-keto-D-gluconic acid, 2-Deoxy-5-keto-D-gluconic acid 6-phosphate, 1-Phosphatidyl-1D-myo-inositol 3,4-bisphosphate, 1D-myo-Inositol 1,4,5,6-tetrakisphosphate, 1-Phosphatidyl-1D-myo-inositol 3,5-bisphosphate, 1-Phosphatidyl-1D-myo-inositol 5-phosphate, 5-Deoxy-D-glucuronate, CDP-1L-myo-inositol, Bis(1L-myo-inositol)-3,1'-phosphate 1-phosphate, 1D-chiro-Inositol, 1-Keto-D-chiro-inositol, 3-Dehydro-scyllo-inosose, 5-Dehydro-L-gluconate
111 M00133_C00315 hsa00330 Arginine and proline metabolism Arginine and proline metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Peptide, S-Adenosyl-L-methionine, Pyruvate, L-Glutamate, Glyoxylate, L-Arginine, L-Ornithine, Urea, Putrescine, L-Proline, Agmatine, Sarcosine, p-Coumaroyl-CoA, Creatine, Spermidine, 4-Aminobutanoate, Feruloyl-CoA, 5-Aminopentanoate, N-Carbamoylputrescine, L-Aspartate 4-semialdehyde, Nitric oxide, 4-Aminobutyraldehyde, Guanidinoacetate, Spermine, D-Proline, Creatinine, Homocarnosine, 1,3-Diaminopropane, 4-Guanidinobutanoate, N-Carbamoylsarcosine, 5-Amino-2-oxopentanoic acid, S-Adenosylmethioninamine, Hydroxyproline, L-Glutamate 5-semialdehyde, N-Acetyl-L-glutamate 5-semialdehyde, Nopaline, Phosphocreatine, N-Methylhydantoin, 4-Guanidinobutanal, N-Acetylputrescine, 4-Acetamidobutanoate, 4-Guanidinobutanamide, Phosphoguanidinoacetate, L-Glutamyl 5-phosphate, N2-Succinyl-L-arginine, Norspermidine, N2-Succinyl-L-ornithine, 1-Pyrroline-2-carboxylate, 5-Guanidino-2-oxopentanoate, (S)-1-Pyrroline-5-carboxylate, Octopine, L-1-Pyrroline-3-hydroxy-5-carboxylate, trans-3-Hydroxy-L-proline, N-Succinyl-L-glutamate, N-Succinyl-L-glutamate 5-semialdehyde, N(omega)-Hydroxyarginine, N4-Acetylaminobutanal, L-4-Hydroxyglutamate semialdehyde, L-Arginine phosphate, (4R)-4-Hydroxy-2-oxoglutarate, L-erythro-4-Hydroxyglutamate, Feruloylputrescine, gamma-L-Glutamylputrescine, gamma-Glutamyl-gamma-aminobutyraldehyde, 4-(L-gamma-Glutamylamino)butanoate, Carboxyspermidine, Carboxynorspermidine, p-Coumaroylputrescine, cis-3-Hydroxy-L-proline, N1-(3-Aminopropyl)agmatine, N1-[(S)-3-amino-3-carboxypropyl]agmatine
112 M00134_C00134 hsa00330 Arginine and proline metabolism Arginine and proline metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Peptide, S-Adenosyl-L-methionine, Pyruvate, L-Glutamate, Glyoxylate, L-Arginine, L-Ornithine, Urea, Putrescine, L-Proline, Agmatine, Sarcosine, p-Coumaroyl-CoA, Creatine, Spermidine, 4-Aminobutanoate, Feruloyl-CoA, 5-Aminopentanoate, N-Carbamoylputrescine, L-Aspartate 4-semialdehyde, Nitric oxide, 4-Aminobutyraldehyde, Guanidinoacetate, Spermine, D-Proline, Creatinine, Homocarnosine, 1,3-Diaminopropane, 4-Guanidinobutanoate, N-Carbamoylsarcosine, 5-Amino-2-oxopentanoic acid, S-Adenosylmethioninamine, Hydroxyproline, L-Glutamate 5-semialdehyde, N-Acetyl-L-glutamate 5-semialdehyde, Nopaline, Phosphocreatine, N-Methylhydantoin, 4-Guanidinobutanal, N-Acetylputrescine, 4-Acetamidobutanoate, 4-Guanidinobutanamide, Phosphoguanidinoacetate, L-Glutamyl 5-phosphate, N2-Succinyl-L-arginine, Norspermidine, N2-Succinyl-L-ornithine, 1-Pyrroline-2-carboxylate, 5-Guanidino-2-oxopentanoate, (S)-1-Pyrroline-5-carboxylate, Octopine, L-1-Pyrroline-3-hydroxy-5-carboxylate, trans-3-Hydroxy-L-proline, N-Succinyl-L-glutamate, N-Succinyl-L-glutamate 5-semialdehyde, N(omega)-Hydroxyarginine, N4-Acetylaminobutanal, L-4-Hydroxyglutamate semialdehyde, L-Arginine phosphate, (4R)-4-Hydroxy-2-oxoglutarate, L-erythro-4-Hydroxyglutamate, Feruloylputrescine, gamma-L-Glutamylputrescine, gamma-Glutamyl-gamma-aminobutyraldehyde, 4-(L-gamma-Glutamylamino)butanoate, Carboxyspermidine, Carboxynorspermidine, p-Coumaroylputrescine, cis-3-Hydroxy-L-proline, N1-(3-Aminopropyl)agmatine, N1-[(S)-3-amino-3-carboxypropyl]agmatine
113 M00135_C00334 hsa00330 Arginine and proline metabolism Arginine and proline metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism NA Peptide, S-Adenosyl-L-methionine, Pyruvate, L-Glutamate, Glyoxylate, L-Arginine, L-Ornithine, Urea, Putrescine, L-Proline, Agmatine, Sarcosine, p-Coumaroyl-CoA, Creatine, Spermidine, 4-Aminobutanoate, Feruloyl-CoA, 5-Aminopentanoate, N-Carbamoylputrescine, L-Aspartate 4-semialdehyde, Nitric oxide, 4-Aminobutyraldehyde, Guanidinoacetate, Spermine, D-Proline, Creatinine, Homocarnosine, 1,3-Diaminopropane, 4-Guanidinobutanoate, N-Carbamoylsarcosine, 5-Amino-2-oxopentanoic acid, S-Adenosylmethioninamine, Hydroxyproline, L-Glutamate 5-semialdehyde, N-Acetyl-L-glutamate 5-semialdehyde, Nopaline, Phosphocreatine, N-Methylhydantoin, 4-Guanidinobutanal, N-Acetylputrescine, 4-Acetamidobutanoate, 4-Guanidinobutanamide, Phosphoguanidinoacetate, L-Glutamyl 5-phosphate, N2-Succinyl-L-arginine, Norspermidine, N2-Succinyl-L-ornithine, 1-Pyrroline-2-carboxylate, 5-Guanidino-2-oxopentanoate, (S)-1-Pyrroline-5-carboxylate, Octopine, L-1-Pyrroline-3-hydroxy-5-carboxylate, trans-3-Hydroxy-L-proline, N-Succinyl-L-glutamate, N-Succinyl-L-glutamate 5-semialdehyde, N(omega)-Hydroxyarginine, N4-Acetylaminobutanal, L-4-Hydroxyglutamate semialdehyde, L-Arginine phosphate, (4R)-4-Hydroxy-2-oxoglutarate, L-erythro-4-Hydroxyglutamate, Feruloylputrescine, gamma-L-Glutamylputrescine, gamma-Glutamyl-gamma-aminobutyraldehyde, 4-(L-gamma-Glutamylamino)butanoate, Carboxyspermidine, Carboxynorspermidine, p-Coumaroylputrescine, cis-3-Hydroxy-L-proline, N1-(3-Aminopropyl)agmatine, N1-[(S)-3-amino-3-carboxypropyl]agmatine
114 M00141_R00943 hsa00670 One carbon pool by folate One carbon pool by folate - Homo sapiens (human) Metabolism; Metabolism of cofactors and vitamins NA Tetrahydrofolate, 5,10-Methylenetetrahydrofolate, 10-Formyltetrahydrofolate, Dihydrofolate, 5-Methyltetrahydrofolate, 5,10-Methenyltetrahydrofolate, Folate, 5-Formiminotetrahydrofolate, Folinic acid
115 M00338_C00097 hsa01230 Biosynthesis of amino acids Biosynthesis of amino acids - Homo sapiens (human) NA This map presents a modular architecture of the biosynthesis pathways of twenty amino acids, which may be viewed as consisting of the core part and its extensions. The core part is the KEGG module for conversion of three-carbon compounds from glyceraldehyde-3P to pyruvate [MD:M00002], together with the pathways around serine and glycine. This KEGG module is the most conserved one in the KEGG MODULE database and is found in almost all the completely sequenced genomes. The extensions are the pathways containing the reaction modules RM001, RM033, RM032, and RM002 for biosynthesis of branched-chain amino acids (left) and basic amino acids (bottom), and the pathways for biosynthesis of histidine and aromatic amino acids (top right). It is interesting to note that the so-called essential amino acids that cannot be synthesized in human and other organisms generally appear in these extensions. Furthermore, the bottom extension of basic amino acids appears to be most divergent containing multiple pathways for lysine biosynthesis and multiple gene sets for arginine biosynthesis. NA
116 M00338_C00097 hsa00270 Cysteine and methionine metabolism Cysteine and methionine metabolism - Homo sapiens (human) Metabolism; Amino acid metabolism Cysteine and methionine are sulfur-containing amino acids. Cysteine is synthesized from serine through different pathways in different organism groups. In bacteria and plants, cysteine is converted from serine (via acetylserine) by transfer of hydrogen sulfide [MD:M00021]. In animals, methionine-derived homocysteine is used as sulfur source and its condensation product with serine (cystathionine) is converted to cysteine [MD:M00338]. Cysteine is metabolized to pyruvate in multiple routes. Methionine is an essential amino acid, which animals cannot synthesize. In bacteria and plants, methionine is synthesized from aspartate [MD:M00017]. S-Adenosylmethionine (SAM), synthesized from methionine and ATP, is a methyl group donor in many important transfer reactions including DNA methylation for regulation of gene expression. SAM may also be used to regenerate methionine in the methionine salvage pathway [MD:M00034]. S-Adenosyl-L-methionine, S-Adenosyl-L-homocysteine, Pyruvate, L-Alanine, L-Aspartate, Glutathione, Sulfate, L-Serine, L-Methionine, Sulfite, L-Cysteine, 2-Oxobutanoate, L-Homocysteine, 5'-Methylthioadenosine, 3-Phospho-D-glycerate, L-Homoserine, Hydrogen sulfide, Methanethiol, L-Aspartate 4-semialdehyde, L-Cystine, L-Cysteate, 3-Sulfino-L-alanine, D-Cysteine, Mercaptopyruvate, O-Acetyl-L-serine, O-Phospho-L-serine, O-Acetyl-L-homoserine, O-Phospho-L-homoserine, O-Succinyl-L-homoserine, S-Adenosylmethioninamine, 4-Methylthio-2-oxobutanoic acid, 1-Aminocyclopropane-1-carboxylate, L-Homocystine, Thiocysteine, Dehydroalanine, L-Cystathionine, (S)-2-Aminobutanoate, L-Methionine S-oxide, 4-Phospho-L-aspartate, 5-Methylthio-D-ribose, N-Formylmethionine, 3-Phosphonooxypyruvate, S-Inosyl-L-homocysteine, S-Ribosyl-L-homocysteine, S-Methyl-5-thio-D-ribose 1-phosphate, S-Methyl-5-thio-D-ribulose 1-phosphate, Nicotianamine, Aminoacyl-L-methionine, S-Glutathionyl-L-cysteine, 3-Sulfinylpyruvate, 3-Sulfopyruvate, 3-Mercaptolactate, S-Sulfo-L-cysteine, Ethylene, 3-(Methylthio)propanoate, Sulfur dioxide, 1-Deoxy-D-xylulose 5-phosphate, HSO3-, (S)-3-Sulfolactate, (2R)-3-Sulfolactate, 1,2-Dihydroxy-5-(methylthio)pent-1-en-3-one, 2,3-Diketo-5-methylthiopentyl-1-phosphate, 2-Hydroxy-3-keto-5-methylthiopentenyl-1-phosphate, N-Acyl-L-homoserine lactone, 5'-S-Methyl-5'-thioinosine, gamma-L-Glutamyl-L-2-aminobutyrate, Ophthalmate, S-Methyl-1-thio-D-xylulose 5-phosphate
117 M00367_C00353 hsa00900 Terpenoid backbone biosynthesis Terpenoid backbone biosynthesis - Homo sapiens (human) Metabolism; Metabolism of terpenoids and polyketides Terpenoids, also known as isoprenoids, are a large class of natural products consisting of isoprene (C5) units. There are two biosynthetic pathways, the mevalonate pathway [MD:M00095] and the non-mevalonate pathway or the MEP/DOXP pathway [MD:M00096], for the terpenoid building blocks: isopentenyl diphosphate (IPP) and dimethylallyl diphosphate (DMAPP). The action of prenyltransferases then generates higher-order building blocks: geranyl diphosphate (GPP), farsenyl diphosphate (FPP), and geranylgeranyl diphosphate (GGPP), which are the precursors of monoterpenoids (C10), sesquiterpenoids (C15), and diterpenoids (C20), respectively. Condensation of these building blocks gives rise to the precursors of sterols (C30) and carotenoids (C40). The MEP/DOXP pathway is absent in higher animals and fungi, but in green plants the MEP/DOXP and mevalonate pathways co-exist in separate cellular compartments. The MEP/DOXP pathway, operating in the plastids, is responsible for the formation of essential oil monoterpenes and linalyl acetate, some sesquiterpenes, diterpenes, and carotenoids and phytol. The mevalonate pathway, operating in the cytosol, gives rise to triterpenes, sterols, and most sesquiterpenes. Pyruvate, Acetyl-CoA, D-Glyceraldehyde 3-phosphate, Isopentenyl diphosphate, Dimethylallyl diphosphate, Acetoacetyl-CoA, Geranyl diphosphate, Geranylgeranyl diphosphate, (S)-3-Hydroxy-3-methylglutaryl-CoA, (R)-Mevalonate, trans,trans-Farnesyl diphosphate, (R)-5-Phosphomevalonate, (2E,6E)-Farnesol, (R)-5-Diphosphomevalonate, all-trans-Hexaprenyl diphosphate, cis-1,4-Polyisoprene, 2-trans,6-trans-Farnesal, all-trans-Nonaprenyl diphosphate, all-trans-Octaprenyl diphosphate, all-trans-Heptaprenyl diphosphate, Protein C-terminal S-farnesyl-L-cysteine, di-trans,poly-cis-Undecaprenyl diphosphate, Protein C-terminal S-farnesyl-L-cysteine methyl ester, Phytyl diphosphate, ditrans,polycis-Polyprenyl diphosphate, Polyprenol, 2-C-Methyl-D-erythritol 4-phosphate, 4-(Cytidine 5'-diphospho)-2-C-methyl-D-erythritol, 2-Phospho-4-(cytidine 5'-diphospho)-2-C-methyl-D-erythritol, 1-Deoxy-D-xylulose 5-phosphate, 2-C-Methyl-D-erythritol 2,4-cyclodiphosphate, 1-Hydroxy-2-methyl-2-butenyl 4-diphosphate, Isoprene, 2-cis,6-trans-Farnesyl diphosphate, all-trans-Decaprenyl diphosphate, trans,octacis-Decaprenyl diphosphate, Farnesylcysteine, 2-cis,6-cis-Farnesyl diphosphate, tritrans,heptacis-Undecaprenyl diphosphate, trans,polycis-Polyprenyl diphosphate, S-Farnesyl protein, trans,trans-Farnesyl phosphate, Isopentenyl phosphate, (R)-Mevalonate 3-phosphate, (R)-Mevalonate 3,5-bisphosphate, trans-Anhydromevalonate 5-phosphate
118 M00415_C20876 hsa01212 Fatty acid metabolism Fatty acid metabolism - Homo sapiens (human) NA NA NA
119 M00415_C20876 hsa00062 Fatty acid elongation Fatty acid elongation - Homo sapiens (human) Metabolism; Lipid metabolism NA Acetyl-CoA, Acyl-CoA, Malonyl-CoA, Butanoyl-CoA, Palmitoyl-CoA, Fatty acid, Hexadecanoic acid, 3-Oxoacyl-CoA, 2,3-Dehydroacyl-CoA, Long-chain fatty acid, (3S)-3-Hydroxyacyl-CoA, trans-2,3-Dehydroacyl-CoA, Lauroyl-CoA, Octanoyl-CoA, Tetradecanoyl-CoA, Long-chain acyl-CoA, 2-trans-Dodecenoyl-CoA, (S)-3-Hydroxyhexadecanoyl-CoA, 3-Oxopalmitoyl-CoA, (S)-3-Hydroxytetradecanoyl-CoA, 3-Oxotetradecanoyl-CoA, (S)-3-Hydroxydodecanoyl-CoA, 3-Oxododecanoyl-CoA, (S)-Hydroxydecanoyl-CoA, 3-Oxodecanoyl-CoA, (S)-3-Hydroxyoctanoyl-CoA, 3-Oxooctanoyl-CoA, (S)-Hydroxyhexanoyl-CoA, 3-Oxohexanoyl-CoA, Hexanoyl-CoA, trans-Hex-2-enoyl-CoA, trans-Hexadec-2-enoyl-CoA, trans-Tetradec-2-enoyl-CoA, Decanoyl-CoA, trans-Dec-2-enoyl-CoA, trans-Oct-2-enoyl-CoA, Very-long-chain acyl-CoA, Very-long-chain 3-oxoacyl-CoA, Very-long-chain (3R)-3-hydroxyacyl-CoA, Very-long-chain trans-2,3-dehydroacyl-CoA
120 M00549_C00029 hsa00520 sugar and nucleotide sugar metabolism Amino sugar and nucleotide sugar metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA UDP-glucose, D-Glucose, UDP-N-acetyl-alpha-D-glucosamine, UDP-alpha-D-galactose, D-Fructose 6-phosphate, D-Fructose, GDP-mannose, D-Glucose 1-phosphate, CMP-N-acetylneuraminate, N-Acetyl-D-glucosamine, D-Mannose, UDP-glucuronate, D-Xylose, UDP-D-xylose, D-Glucuronate, UDP-N-acetyl-D-galactosamine, D-Arabinose, L-Arabinose, alpha-D-Glucose, N-Acetylneuraminate, D-Mannose 6-phosphate, GDP-L-fucose, D-Glucosamine, D-Galacturonate, D-Glucosamine 6-phosphate, N-Acetyl-D-glucosamine 6-phosphate, GDP-glucose, alpha-D-Galactose 1-phosphate, Chitin, ADP-glucose, CDP-glucose, UDP-D-galacturonate, D-Mannose 1-phosphate, N-Acetyl-D-mannosamine, alpha-D-Glucose 6-phosphate, Pectin, Chitosan, UDP-L-arabinose, GDP-D-mannuronate, alpha-D-Galactose, Ferricytochrome b5, Ferrocytochrome b5, L-Fucose, UDP-N-acetylmuramate, N-Acetyl-D-galactosamine, UDP-N-acetyl-D-mannosamine, CDP-4-dehydro-6-deoxy-D-glucose, GDP-4-dehydro-6-deoxy-D-mannose, UDP-apiose, Chitobiose, CDP-abequose, UDP-L-rhamnose, GDP-L-galactose, 1,4-beta-D-Xylan, Arabinan, N-Acetylmuramate, GDP-6-deoxy-D-talose, L-Fucose 1-phosphate, GDP-6-deoxy-D-mannose, N-Glycoloyl-neuraminate, CDP-3,6-dideoxy-D-glucose, CDP-3,6-dideoxy-D-mannose, CMP-N-glycoloylneuraminate, UDP-alpha-D-galactofuranose, beta-L-Arabinose 1-phosphate, 1-Phospho-alpha-D-galacturonate, UDP-4-dehydro-6-deoxy-D-glucose, N-Acetyl-D-mannosamine 6-phosphate, CDP-4-dehydro-3,6-dideoxy-D-glucose, N-Acetyl-alpha-D-glucosamine 1-phosphate, UDP-N-acetyl-2-amino-2-deoxy-D-glucuronate, UDP-2-acetamido-4-dehydro-2,6-dideoxyglucose, UDP-2-acetamido-4-amino-2,4,6-trideoxy-alpha-D-glucose, UDP-N-acetyl-3-(1-carboxyvinyl)-D-glucosamine, D-Glucuronate 1-phosphate, D-Glucosaminide, alpha-D-Glucosamine 1-phosphate, UDP-N-acetyl-D-mannosaminouronate, N-Acetylneuraminate 9-phosphate, D-Galactosamine 6-phosphate, UDP-6-sulfoquinovose, UDP-N-acetyl-D-galactosaminuronic acid, GDP-L-gulose, UDP-L-Ara4N, UDP-L-Ara4FN, UDP-L-Ara4O, Undecaprenyl phosphate alpha-L-Ara4FN, Undecaprenyl phosphate alpha-L-Ara4N, alpha-D-Arabinopyranose 1-phosphate, N-Acetylmuramic acid 6-phosphate, GDP-D-arabinopyranose, CDP-4-dehydro-3,6-dideoxy-D-glucose epimer, CDP-ascarylose, UDP-4-keto-rhamnose, N-Acetyl-alpha-D-galactosamine 1-phosphate, UDP-L-arabinofuranose, UDP-2,3-diacetamido-2,3-dideoxy-alpha-D-glucuronate, UDP-2,3-diacetamido-2,3-dideoxy-alpha-D-mannuronate, 1,6-Anhydro-N-acetyl-beta-muramate, UDP-2-acetamido-2,6-dideoxy-beta-L-arabino-hexos-4-ulose, UDP-4-amino-4,6-dideoxy-N-acetyl-beta-L-altrosamine, UDP-2,4-bis(acetamido)-2,4,6-trideoxy-beta-L-altropyranose, 2,4-Bis(acetamido)-2,4,6-trideoxy-beta-L-altropyranose, Pseudaminic acid, CMP-pseudaminic acid, UDP-N,N'-diacetylbacillosamine, UDP-2-acetamido-3-amino-2,3-dideoxy-alpha-D-glucuronate, UDP-2-acetamido-2-deoxy-alpha-D-ribo-hex-3-uluronate, N,N'-Diacetyllegionaminate, CMP-N,N'-diacetyllegionaminate, 2,4-Diacetamido-2,4,6-trideoxy-D-mannopyranose, GDP-4-amino-4,6-dideoxy-alpha-D-mannose, GDP-4-acetamido-4,6-dideoxy-alpha-D-mannose, 3-Deoxy-D-glycero-D-galacto-non-2-ulopyranosonate, N-Acetylmuramic acid alpha-1-phosphate, 3-Deoxy-D-glycero-D-galacto-non-2-ulopyranosonate 9-phosphate, CMP-3-deoxy-D-glycero-D-galacto-non-2-ulopyranosonate, alpha-D-Galactosamine 1-phosphate, UDP-N-acetyl-alpha-D-xylosamine
121 M00554_C00052 hsa00520 sugar and nucleotide sugar metabolism Amino sugar and nucleotide sugar metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA UDP-glucose, D-Glucose, UDP-N-acetyl-alpha-D-glucosamine, UDP-alpha-D-galactose, D-Fructose 6-phosphate, D-Fructose, GDP-mannose, D-Glucose 1-phosphate, CMP-N-acetylneuraminate, N-Acetyl-D-glucosamine, D-Mannose, UDP-glucuronate, D-Xylose, UDP-D-xylose, D-Glucuronate, UDP-N-acetyl-D-galactosamine, D-Arabinose, L-Arabinose, alpha-D-Glucose, N-Acetylneuraminate, D-Mannose 6-phosphate, GDP-L-fucose, D-Glucosamine, D-Galacturonate, D-Glucosamine 6-phosphate, N-Acetyl-D-glucosamine 6-phosphate, GDP-glucose, alpha-D-Galactose 1-phosphate, Chitin, ADP-glucose, CDP-glucose, UDP-D-galacturonate, D-Mannose 1-phosphate, N-Acetyl-D-mannosamine, alpha-D-Glucose 6-phosphate, Pectin, Chitosan, UDP-L-arabinose, GDP-D-mannuronate, alpha-D-Galactose, Ferricytochrome b5, Ferrocytochrome b5, L-Fucose, UDP-N-acetylmuramate, N-Acetyl-D-galactosamine, UDP-N-acetyl-D-mannosamine, CDP-4-dehydro-6-deoxy-D-glucose, GDP-4-dehydro-6-deoxy-D-mannose, UDP-apiose, Chitobiose, CDP-abequose, UDP-L-rhamnose, GDP-L-galactose, 1,4-beta-D-Xylan, Arabinan, N-Acetylmuramate, GDP-6-deoxy-D-talose, L-Fucose 1-phosphate, GDP-6-deoxy-D-mannose, N-Glycoloyl-neuraminate, CDP-3,6-dideoxy-D-glucose, CDP-3,6-dideoxy-D-mannose, CMP-N-glycoloylneuraminate, UDP-alpha-D-galactofuranose, beta-L-Arabinose 1-phosphate, 1-Phospho-alpha-D-galacturonate, UDP-4-dehydro-6-deoxy-D-glucose, N-Acetyl-D-mannosamine 6-phosphate, CDP-4-dehydro-3,6-dideoxy-D-glucose, N-Acetyl-alpha-D-glucosamine 1-phosphate, UDP-N-acetyl-2-amino-2-deoxy-D-glucuronate, UDP-2-acetamido-4-dehydro-2,6-dideoxyglucose, UDP-2-acetamido-4-amino-2,4,6-trideoxy-alpha-D-glucose, UDP-N-acetyl-3-(1-carboxyvinyl)-D-glucosamine, D-Glucuronate 1-phosphate, D-Glucosaminide, alpha-D-Glucosamine 1-phosphate, UDP-N-acetyl-D-mannosaminouronate, N-Acetylneuraminate 9-phosphate, D-Galactosamine 6-phosphate, UDP-6-sulfoquinovose, UDP-N-acetyl-D-galactosaminuronic acid, GDP-L-gulose, UDP-L-Ara4N, UDP-L-Ara4FN, UDP-L-Ara4O, Undecaprenyl phosphate alpha-L-Ara4FN, Undecaprenyl phosphate alpha-L-Ara4N, alpha-D-Arabinopyranose 1-phosphate, N-Acetylmuramic acid 6-phosphate, GDP-D-arabinopyranose, CDP-4-dehydro-3,6-dideoxy-D-glucose epimer, CDP-ascarylose, UDP-4-keto-rhamnose, N-Acetyl-alpha-D-galactosamine 1-phosphate, UDP-L-arabinofuranose, UDP-2,3-diacetamido-2,3-dideoxy-alpha-D-glucuronate, UDP-2,3-diacetamido-2,3-dideoxy-alpha-D-mannuronate, 1,6-Anhydro-N-acetyl-beta-muramate, UDP-2-acetamido-2,6-dideoxy-beta-L-arabino-hexos-4-ulose, UDP-4-amino-4,6-dideoxy-N-acetyl-beta-L-altrosamine, UDP-2,4-bis(acetamido)-2,4,6-trideoxy-beta-L-altropyranose, 2,4-Bis(acetamido)-2,4,6-trideoxy-beta-L-altropyranose, Pseudaminic acid, CMP-pseudaminic acid, UDP-N,N'-diacetylbacillosamine, UDP-2-acetamido-3-amino-2,3-dideoxy-alpha-D-glucuronate, UDP-2-acetamido-2-deoxy-alpha-D-ribo-hex-3-uluronate, N,N'-Diacetyllegionaminate, CMP-N,N'-diacetyllegionaminate, 2,4-Diacetamido-2,4,6-trideoxy-D-mannopyranose, GDP-4-amino-4,6-dideoxy-alpha-D-mannose, GDP-4-acetamido-4,6-dideoxy-alpha-D-mannose, 3-Deoxy-D-glycero-D-galacto-non-2-ulopyranosonate, N-Acetylmuramic acid alpha-1-phosphate, 3-Deoxy-D-glycero-D-galacto-non-2-ulopyranosonate 9-phosphate, CMP-3-deoxy-D-glycero-D-galacto-non-2-ulopyranosonate, alpha-D-Galactosamine 1-phosphate, UDP-N-acetyl-alpha-D-xylosamine
122 M00554_C00052 hsa00052 metabolism Galactose metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA UDP-glucose, D-Glucose, UDP-alpha-D-galactose, D-Fructose 6-phosphate, Sucrose, D-Fructose, D-Glucose 1-phosphate, Glycerone phosphate, Glycerol, D-Glyceraldehyde 3-phosphate, D-Galactose, myo-Inositol, D-Mannose, Lactose, alpha-D-Glucose, alpha-D-Galactose 1-phosphate, Raffinose, D-Glyceraldehyde, alpha-D-Glucose 6-phosphate, D-Sorbitol, D-Tagatose, D-Galactonate, alpha-D-Galactose, D-Tagatose 6-phosphate, D-Galactose 6-phosphate, N-Acetyl-D-galactosamine, 2-Dehydro-3-deoxy-D-galactonate, alpha-D-Galactosyl-(1->3)-1D-myo-inositol, 2-Dehydro-3-deoxy-6-phospho-D-galactonate, Stachyose, Galactitol, D-Galactosamine, D-Galactono-1,5-lactone, D-Galactono-1,4-lactone, UDP-alpha-D-galactofuranose, D-Tagatose 1,6-bisphosphate, Lactose 6'-phosphate, Melibiitol, Epimelibiose, 3-beta-D-Galactosyl-sn-glycerol, Melibiose, D-Gal alpha 1->6D-Gal alpha 1->6D-Glucose, Galactan, Galactitol 1-phosphate, N-Acetyl-D-galactosamine 6-phosphate, D-Galactosamine 6-phosphate
123 M00632_C00103 hsa00052 metabolism Galactose metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA UDP-glucose, D-Glucose, UDP-alpha-D-galactose, D-Fructose 6-phosphate, Sucrose, D-Fructose, D-Glucose 1-phosphate, Glycerone phosphate, Glycerol, D-Glyceraldehyde 3-phosphate, D-Galactose, myo-Inositol, D-Mannose, Lactose, alpha-D-Glucose, alpha-D-Galactose 1-phosphate, Raffinose, D-Glyceraldehyde, alpha-D-Glucose 6-phosphate, D-Sorbitol, D-Tagatose, D-Galactonate, alpha-D-Galactose, D-Tagatose 6-phosphate, D-Galactose 6-phosphate, N-Acetyl-D-galactosamine, 2-Dehydro-3-deoxy-D-galactonate, alpha-D-Galactosyl-(1->3)-1D-myo-inositol, 2-Dehydro-3-deoxy-6-phospho-D-galactonate, Stachyose, Galactitol, D-Galactosamine, D-Galactono-1,5-lactone, D-Galactono-1,4-lactone, UDP-alpha-D-galactofuranose, D-Tagatose 1,6-bisphosphate, Lactose 6'-phosphate, Melibiitol, Epimelibiose, 3-beta-D-Galactosyl-sn-glycerol, Melibiose, D-Gal alpha 1->6D-Gal alpha 1->6D-Glucose, Galactan, Galactitol 1-phosphate, N-Acetyl-D-galactosamine 6-phosphate, D-Galactosamine 6-phosphate
124 M00741_C00091 hsa01200 Carbon metabolism Carbon metabolism - Homo sapiens (human) NA Carbon metabolism is the most basic aspect of life. This map presents an overall view of central carbon metabolism, where the number of carbons is shown for each compound denoted by a circle, excluding a cofactor (CoA, CoM, THF, or THMPT) that is replaced by an asterisk. The map contains carbon utilization pathways of glycolysis (map00010), pentose phosphate pathway (map00030), and citrate cycle (map00020), and six known carbon fixation pathways (map00710 and map00720) as well as some pathways of methane metabolism (map00680). The six carbon fixation pathways are: (1) reductive pentose phosphate cycle (Calvin cycle) in plants and cyanobacteria that perform oxygenic photosynthesis, (2) reductive citrate cycle in photosynthetic green sulfur bacteria and some chemolithoautotrophs, (3) 3-hydroxypropionate bi-cycle in photosynthetic green nonsulfur bacteria, two variants of 4-hydroxybutyrate pathways in Crenarchaeota called (4) hydroxypropionate-hydroxybutyrate cycle and (5) dicarboxylate-hydroxybutyrate cycle, and (6) reductive acetyl-CoA pathway in methanogenic bacteria. NA
125 M00741_C00091 hsa00280 Valine, leucine and isoleucine degradation Valine, leucine and isoleucine degradation - Homo sapiens (human) Metabolism; Amino acid metabolism NA Acetyl-CoA, Thiamin diphosphate, Succinyl-CoA, Propanoyl-CoA, L-Leucine, 3-Methyl-2-oxobutanoic acid, Acetoacetate, L-Valine, 4-Methyl-2-oxopentanoate, Acetoacetyl-CoA, 2-Methyl-3-oxopropanoate, (S)-3-Hydroxy-3-methylglutaryl-CoA, L-Isoleucine, 2-Methylpropanoyl-CoA, (S)-3-Methyl-2-oxopentanoic acid, (S)-Methylmalonyl-CoA, (R)-3-Amino-2-methylpropanoate, (R)-Methylmalonyl-CoA, Methylmalonate, 3-Methylbutanoyl-CoA, 3-Methylcrotonyl-CoA, 3-Methylglutaconyl-CoA, L-3-Aminoisobutanoate, 2-Methylacetoacetyl-CoA, 2-Methylbut-2-enoyl-CoA, 2-Methylprop-2-enoyl-CoA, (2S,3S)-3-Hydroxy-2-methylbutanoyl-CoA, Branched chain fatty acid, 3-Hydroxyisovaleryl-CoA, (S)-3-Hydroxyisobutyryl-CoA, (S)-3-Hydroxyisobutyrate, (S)-Methylmalonate semialdehyde, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, 3-Methyl-1-hydroxybutyl-ThPP, [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(3-methylbutanoyl)dihydrolipoyllysine, 2-Methyl-1-hydroxypropyl-ThPP, [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine, 2-Methyl-1-hydroxybutyl-ThPP, [Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylbutanoyl)dihydrolipoyllysine, (S)-2-Methylbutanoyl-CoA, 3-Hydroxyisovalerate
126 M00741_C00091 hsa00630 Glyoxylate and dicarboxylate metabolism Glyoxylate and dicarboxylate metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA Oxygen, CO2, Ammonia, Pyruvate, Acetyl-CoA, L-Glutamate, 2-Oxoglutarate, Hydrogen peroxide, Acetate, Oxaloacetate, Glycine, Succinate, Glyoxylate, Formate, L-Glutamine, L-Serine, Succinyl-CoA, Propanoyl-CoA, Glycerone phosphate, Butanoyl-CoA, (S)-Malate, Citrate, Glycolate, Hydroxypyruvate, 3-Phospho-D-glycerate, Oxalate, D-Glycerate, Glycolaldehyde, Isocitrate, Oxalyl-CoA, Acetoacetyl-CoA, cis-Aconitate, meso-Tartaric acid, 2-Phospho-D-glycerate, (S)-Methylmalonyl-CoA, Formyl-CoA, Crotonoyl-CoA, Pentanoyl-CoA, (R,R)-Tartaric acid, Dihydroxyfumarate, 2-Phosphoglycolate, 4-Hydroxy-2-oxoglutarate, 2-Hydroxy-3-oxopropanoate, D-Ribulose 1,5-bisphosphate, (R)-Methylmalonyl-CoA, Ethylene glycol, Mesaconate, 3-Ethylmalate, 3-Oxalomalate, 3-Propylmalate, Formyl phosphate, 2-Hydroxy-3-oxoadipate, 2-Hydroxy-3-oxosuccinate, trans-2,3-Epoxysuccinate, (R)-3-Hydroxybutanoyl-CoA, L-threo-3-Methylaspartate, L-Malyl-CoA, L-erythro-3-Methylmalyl-CoA, 2-Methylfumaryl-CoA, N-Formylderivatives, (2S)-Ethylmalonyl-CoA, (2S)-Methylsuccinyl-CoA, (2R)-Ethylmalonyl-CoA, D-Ribulose 1-phosphate
127 M00741_C00091 hsa00640 Propanoate metabolism Propanoate metabolism - Homo sapiens (human) Metabolism; Carbohydrate metabolism NA Acetyl-CoA, Oxaloacetate, Succinate, Thiamin diphosphate, Malonyl-CoA, Succinyl-CoA, beta-Alanine, Propanoyl-CoA, 2-Oxobutanoate, Glycerone phosphate, Propanoate, (S)-Lactate, 3-Oxopropanoate, (S)-Lactaldehyde, Propanal, Acrylic acid, Methylglyoxal, Propane-1,2-diol, (S)-Methylmalonyl-CoA, Propynoate, Lactoyl-CoA, Propenoyl-CoA, 3-Hydroxypropanoate, (R)-Methylmalonyl-CoA, Methylmalonate, 2-Methylcitrate, beta-Alanyl-CoA, Propanoyl phosphate, (Z)-But-2-ene-1,2,3-tricarboxylate, (2S,3R)-3-Hydroxybutane-1,2,3-tricarboxylate, Hydroxyacetone, 3-Hydroxypropionyl-CoA, Propane-1-ol, Propionyladenylate, 2-Hydroxybutanoic acid, 3-Oxopropionyl-CoA, (S)-Methylmalonate semialdehyde, Enzyme N6-(lipoyl)lysine, Enzyme N6-(dihydrolipoyl)lysine, 2-(alpha-Hydroxypropyl)thiamine diphosphate, Enzyme N6-(S-propyldihydrolipoyl)lysine, 2-Methyl-trans-aconitate