期刊
TRENDS IN MOLECULAR MEDICINE
卷 7, 期 4, 页码 157-162出版社
ELSEVIER SCI LTD
DOI: 10.1016/S1471-4914(01)01955-4
关键词
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The neurofibromatoses NF1 and NF2 are inherited cancer predisposition syndromes in which affected individuals are prone to development of mostly benign, hut occasionally malignant, tumors. The NF7 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.
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