Tumorigenesis in neurofibromatosis: new insights and potential therapies.
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.
Reed
N
N
Department of Neurology, Pediatrics and Genetics, Washington University School of Medicine, Box 8111, 660 S. Euclid Avenue, St Louis, MO 63110, USA.
Gutmann
D H
DH
D002455:Cell Division; D002465:Cell Movement; D006801:Humans; D008565:Membrane Proteins / Q000378:metabolism; D008954:Models, Biological; D009419:Nerve Tissue Proteins / Q000737:chemistry; D009456:Neurofibromatosis 1 / Q000188:drug therapy* / Q000209:etiology* / Q000235:genetics; D016518:Neurofibromatosis 2 / Q000188:drug therapy* / Q000209:etiology* / Q000235:genetics; D025542:Neurofibromin 1; D025581:Neurofibromin 2; D000090063:Proto-Oncogene Mas; D015398:Signal Transduction; D018631:ras Proteins / Q000378:metabolism
C000715470:MAS1 protein, human; D008565:Membrane Proteins; D009419:Nerve Tissue Proteins; D025542:Neurofibromin 1; D025581:Neurofibromin 2; D000090063:Proto-Oncogene Mas; D018631:ras Proteins