Analysis of the vascular degeneration mechanism in familial vascular dementia CADASIL

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We previously reported that the aggregate-prone property of mutant Notch3 might contribute to a pathogenic mechanism underlying familial vascular dementia CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy). In this study, we searched for low-molecular compounds that decrease the amount of mutant Notch3 aggregates using the mutant Notch3 inducible stable cell lines. Furthermore, we analyzed the morphological and molecular changes by mutant Notch3 using the knock-in mice.