Mouse Models of Neurofibromatosis 1 and 2

• Main Authors: David H. Gutmann, Marco Giovannini
• Format: Article
• Language: English
• Published: Elsevier 2002-01-01
• Series: Neoplasia: An International Journal for Oncology Research
• Subjects: neurofibromin; RAS; tumor suppressor gene; ERM proteins; actin cytoskeleton
• Online Access: http://www.sciencedirect.com/science/article/pii/S1476558602800227

The neurofibromatoses represent two of the most common inherited tumor predisposition syndromes affecting the nervous system. Individuals with neurofibromatosis 1 (NF1) are prone to the development of astrocytomas and peripheral nerve sheath tumors whereas those affected with neurofibromatosis 2 (NF2) develop schwannomas and meningiomas. The development of traditional homozygous knockout mice has provided insights into the roles of the NF1 and NF2 genes during development and in differentiation, but has been less instructive regarding the contribution of NF1 and NF2 dysfunction to the pathogenesis of specific benign and malignant tumors. Recent progress employing novel mouse targeting strategies has begun to illuminate the roles of the NF1 and NF2 gene products in the molecular pathogenesis of NF-associated tumors.