Vascular smooth muscle cell is the primary target of events leading from notch3 mutations to cadasil

CADASIL is an increasingly recognized cause of stroke and dementia in humans. Neuropathological lesions include multiple deep infarcts, severe white matter lesions and diffuse alterations of the small cerebral arteries characterized by major alterations of vascular smooth muscle cells, which ultimately disappear. We recently established that CADASIL is due to mutations in the Notch3 gene.