Mouse Tumor Model for Neurofibromatosis Type 1

• DOI: 10.1126/science.286.5447.2176
• Publication Date: 2002-07-27T09:37:20Z
• Authors: Kristine S. Vogel; Laura J. Klesse; Susana Velasco-Mi...; Kimberly Meyers; Elizabeth J. Rushing; Luis F. Parada
• Keywords: Neurofibromin 1; Malignant Transformation; Heterozygote advantage

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by increased incidence of benign and malignant tumors neural crest origin. Mutations that activate the protooncogene ras, such as loss Nf1, cooperate with inactivating mutations at p53 tumor suppressor gene during transformation. One hundred percent mice harboring null Nf1 alleles in cis synergize to develop soft tissue sarcomas between 3 7 months age. These exhibit heterozygosity both loci express phenotypic traits characteristic derivatives human NF1 malignancies.