NOTCH3 and CADASIL: Mutations in human NOTCH3 are associated with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), a disorder that causes stroke and dementia and is accompanied by the degeneration of vascular smooth muscle cells [43]; adult Notch3 mutant mice display a defect in the maturation of arterial smooth muscle cells [44].