Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited syndrome that leads to dementia. The key features of the disorder are migraine, recurrent subcortical events, mood disorders, and dementia in association with diffuse leukoaraiosis on neuroimaging. The CADASIL or Notch3 gene encodes a transmembrane (TM) receptor exclusively expressed in vascular smooth muscle cells (VSMCs) in adult human tissues. The brain pathology is typical of subcortical vascular dementia with a diffuse leukoencephalopathy and numerous small deep infarcts. This is underlaid by a systemic angiopathy characterized by prominent VSMC alterations in humans as well as in Notch3 transgenic mice. So far, mechanisms of such VSMC destruction are not elucidated and further analysis of the Notch3 pathway and of a more adapted animal model will provide insights into the CADASIL VSMC physiology. The next and most important step is to find preventive treatments for this dramatic condition.

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