Mouse Tumor Model for Neurofibromatosis Type 1

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Science  10 Dec 1999:
Vol. 286, Issue 5447, pp. 2176-2179
DOI: 10.1126/science.286.5447.2176

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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 protooncogeneras, 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.

  • * These authors contributed equally to this work.

  • Present address: Department of Cell Biology and Anatomy, Louisiana State University Medical Center, New Orleans, LA 70112, USA.

  • To whom correspondence should be addressed. E-mail: parada{at}

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