Tumorigenesis in neurofibromatosis: New insights and potential therapies

Nevada Reed, David H. Gutmann

Research output: Contribution to journalReview article

52 Scopus citations

Abstract

The neurofibromatoses NF1 and NF2 are inherited cancer predisposition syndromes in which affected individuals are prone to development of mostly benign, but occasionally malignant, tumors. The NF1 and NF2 genes function as tumor suppressor genes (negative growth regulators), such that their loss of expression predisposes to tumor formation. Neurofibromin, the protein product of the NF1 gene, acts as a negative regulator of the ras proto-oncogene, to reduce cell growth. Merlin, the NF2 gene product, is involved in regulating cell proliferation and motility, and probably plays a role in integrating multiple cell-signaling pathways. By understanding the function of these tumor suppressors, we have a unique opportunity to develop targeted pharmacotherapeutic interventions for these disorders.

Original languageEnglish
Pages (from-to)157-162
Number of pages6
JournalTrends in Molecular Medicine
Volume7
Issue number4
DOIs
StatePublished - Apr 19 2001

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