Article
Peripheral nerve and skeletal muscle involvement in CADASIL.
Acta neuropathologica - 1 Dec 2005
Schröder J M, Züchner S, Dichgans M, Nagy Z, Molnar M J
Abstract excerpt
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by degeneration of vascular smooth muscle cells (VSMC) of nearly all tissues studied so far. The clinical phenotype of CADASIL shows great variability. The disease is caused by mutations of the Notch3 gene encoding the transmembrane receptor Notch3, which is expressed predominantly in VSMC. In...
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