| pmid | 10591653 | ||||||
|---|---|---|---|---|---|---|---|
| title | Mouse tumor model for neurofibromatosis type 1. | ||||||
| authors |
|
||||||
| journal | Science | ||||||
| year | 1999 | ||||||
| full_text_available | false | ||||||
| full_text_extraction_method | html_abstract_only | ||||||
| pmcid | PMC3079436 | ||||||
| doi | 10.1126/science.286.5447.2176 |
Authors: Vogel KS, Klesse LJ, Velasco-Miguel S, Meyers K, Rushing EJ, Parada LF Journal: Science (1999) DOI: 10.1126/science.286.5447.2176 PMC: PMC3079436
- Science. 1999 Dec 10;286(5447):2176-9. doi: 10.1126/science.286.5447.2176.
Mouse tumor model for neurofibromatosis type 1.
Vogel KS(1), Klesse LJ, Velasco-Miguel S, Meyers K, Rushing EJ, Parada LF.
Author information: (1)Center for Developmental Biology and Department of Pathology, University of Texas Southwestern Medical Center, 6000 Harry Hines Blvd., Dallas, TX 75235-9133, USA.
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by increased incidence of benign and malignant tumors of neural crest origin. Mutations that activate the protooncogene ras, such as loss of Nf1, cooperate with inactivating mutations at the p53 tumor suppressor gene during malignant transformation. One hundred percent of mice harboring null Nf1 and p53 alleles in cis synergize to develop soft tissue sarcomas between 3 and 7 months of age. These sarcomas exhibit loss of heterozygosity at both gene loci and express phenotypic traits characteristic of neural crest derivatives and human NF1 malignancies.
DOI: 10.1126/science.286.5447.2176 PMCID: PMC3079436 PMID: 10591653 [Indexed for MEDLINE]
Abstract
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by increased incidence of benign and malignant tumors of neural crest origin. Mutations that activate the protooncogene ras , such as loss of Nf1 , cooperate with inactivating mutations at the p53 tumor suppressor gene during malignant transformation. One hundred percent of mice harboring null Nf1 and p53 alleles in cis synergize to develop soft tissue sarcomas between 3 and 7 months of age. These sarcomas exhibit loss of heterozygosity at both gene loci and express phenotypic traits characteristic of neural crest derivatives and human NF1 malignancies.