References
Query Results -- Details
MGI Accession ID: MGI:1350531
J Number: J:58876
Other Accession IDs:
Title: Mouse models of tumor development in neurofibromatosis type 1.
Authors: Cichowski K; Shih TS; Schmitt E; Santiago S; Reilly K; McLaughlin ME; Bronson RT; Jacks T
Journal: Science
Volume: 286
Issue: 5447
Date: 1999 Dec 10
Year: 1999
Pages: 2172-6
Review Status: Peer Reviewed
Abstract:
Neurofibromatosis type 1 (NF1) is a prevalent familial cancer syndrome resulting from germ line mutations in the NF1 tumor suppressor gene. Hallmark features of the disease are the development of benign peripheral nerve sheath tumors (neurofibromas), which can progress to malignancy. Unlike humans, mice that are heterozygous for a mutation in Nf1 do not develop neurofibromas. However, as described here, chimeric mice composed in part of Nf1-/- cells do, which demonstrates that loss of the wild-type Nf1 allele is rate-limiting in tumor formation. In addition, mice that carry linked germ line mutations in Nf1 and p53 develop malignant peripheral nerve sheath tumors (MPNSTs), which supports a cooperative and causal role for p53 mutations in MPNST development. These two mouse models provide the means to address fundamental aspects of disease development and to test therapeutic strategies.
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