Notch proteins (Notch1-4) are a family of transmembrane receptors that play important roles in development and the determination of cell fate. The Notch proteins have been found to be overexpressed or rearranged in human tumors. Notch3 is a member of the Notch family and is processed similar to Notch1. It is expressed primarily in arterial smooth muscle cells (SMC). Mutations in NOTCH3 have been identified as the underlying cause of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.
