NOTCH3 and Leukoencephalopathy: Altered TGF-β signalling has been found in a related, rare, recessive, small vessel disease CARASIL (cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy), and increased TGF-β signalling has been proposed to reduce VSMC proliferation in CADASIL as a downstream consequence of Notch3 ECD deposition [82].