Mouse tumor model for neurofibromatosis type 1

Kristine S. Vogel, Laura J. Klesse, Susana Velasco-Miguel, Kimberly Meyers, Elizabeth J. Rushing, Luis F. Parada

Research output: Contribution to journalArticle

258 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)2176-2179
Number of pages4
JournalScience
Volume286
Issue number5447
DOIs
StatePublished - Dec 10 1999

ASJC Scopus subject areas

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    Vogel, K. S., Klesse, L. J., Velasco-Miguel, S., Meyers, K., Rushing, E. J., & Parada, L. F. (1999). Mouse tumor model for neurofibromatosis type 1. Science, 286(5447), 2176-2179. https://doi.org/10.1126/science.286.5447.2176