CADASIL is characterized by the accumulation of granular osmiophilic material (GOM) and the extracellular domain of NOTCH3 in the vascular walls, leading to the loss of perivascular cell (vascular smooth muscle cell and pericyte), vascular dysfunction, recurrent lacunar infarcts, cognitive impairments, depressive symptoms, and motor deficits (Kalaria et al., 2004; Chabriat et al., 2009; Dziewulska and Lewandowska, 2012). Here, NOTCH3 is linked to CADASIL.