Although arteriopathies in the tail and, when challenged, cortical vessels (Arboleda-Velasquez et al., 2008) have been observed in knockout mice, important pathologies and hallmarks of CADASIL – including Notch3 ectodomain deposits, leukoencephalopathy and lacunar infarcts – have not been found. This evidence concerns the gene NOTCH3 and Leukoencephalopathy.