Article
Tumors perturbing extracellular matrix biosynthesis. The case of von Recklinghausen's disease.
Pathologie-biologie - 1 Apr 2014
Robert L
Abstract excerpt
This is a short review of neurofibromatosis-1 or von Recklinghausen's disease, due to a loss of function mutation of the gene neurofibromin-1, which normally inhibits the Ras MAPK-pathways. Among its symptoms, the strong oversynthesis of several collagen types designates this disease as producing a deregulation of extracellular matrix biosynthesis involved in tumor formation. Up to about 40% of the skin tumors...
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