The importance of NOTCH3 signalling in VSMCs haemostasis and function has been reported in other cerebrovascular diseases such as stroke, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), and Alzheimer’s disease (AD), all characterized by amyloid beta (Aβ) accumulation in arteries and clinically with cognitive dysfunction [34, 69]. Here, NOTCH3 is linked to early-onset autosomal dominant Alzheimer disease.