NOTCH3 and CADASIL: CADASIL-related mutations exist in exons 2 to 24 of NOTCH3 encoding epidermal growth factor (EGF)-like domains.[8] Abnormal accumulation and deposition of mutated Notch3 protein aggregates on the surface of VSMCs, pericytes, and endothelial cells are the key pathophysiological mechanisms underlying CADASIL.