Article
Transgenic mice expressing mutant Notch3 develop vascular alterations characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.
The American journal of pathology - 1 Jan 2003
Ruchoux Marie Magdeleine, Domenga Valérie, Brulin Peggy, Maciazek Jacqueline, Limol Sylvie, Tournier-Lasserve Elisabeth, Joutel Anne
Abstract excerpt
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an increasingly recognized adult-onset autosomal dominant vascular dementia, caused by highly stereotyped mutations in the Notch3 receptor. CADASIL is a widespread angiopathy characterized by a degeneration of vascular smooth muscle cells (VSMCs) and the abnormal accumulation of electron-dense granular material...
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