Article
Genetic and cellular defects contributing to benign tumor formation in neurofibromatosis type 1.
Human molecular genetics - 12 Apr 2000
Rutkowski J L, Wu K, Gutmann D H, Boyer P J, Legius E
Abstract excerpt
Neurofibromatosis type 1 (NF1) is a common inherited cancer predisposition syndrome. The NF1 gene product, neurofibromin, is hypothesized to function as a tumor suppressor and nearly all NF1 patients develop benign peripheral nerve tumors. These neurofibromas presumably arise from NF1 inactivation in S100(+)Schwann cells, but there is no formal proof for this mechanism. We demonstrate that fibro-blasts isolated...
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