NOTCH3 and Leukoencephalopathy: In the inherited disease CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), mutations in NOTCH3 lead to SMC degeneration or pericyte insufficiency, associated with blood vessel thickening and blood flow blockage (Joutel et al., 1996; Arboleda-Velasquez et al., 2011; Ghosh et al., 2015; Arango-Lievano et al., 2018).