Notch3 mutations underlie CADASIL, a cerebral arteriopathy characterized by cerebrovascular dysfunction, stroke and premature vascular dementia.5 Notch3 mutations cause CADASIL, likely through gain-of-function (GOF) effects over a loss-of-function mechanism.6 The TgNotch3R169C mouse, a transgenic model of GOF Notch3 mutation has been used to study the pathophysiology of Notch3 in CADASIL and other conditions.7,8 Here, we studied this model in PH. This evidence concerns the gene NOTCH3 and stroke disorder.