Mouse Genome Informatics
cn
    Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0

involves: 129 * C57BL/6 * SJL
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• vascular smooth muscle cell abnormalities with intracellular inclusions are seen at 6 months of age

homeostasis/metabolism
• increased susceptibility to ischemic injury seen in Notch3 null mice is not rescued by expression of the mutant human NOTCH3

muscle
• vascular smooth muscle cell abnormalities with intracellular inclusions are seen at 6 months of age

nervous system
• increased susceptibility to ischemic injury seen in Notch3 null mice is not rescued by expression of the mutant human NOTCH3

Mouse Models of Human Disease
OMIM IDRef(s)
Cerebral Arteriopathy, Autosomal Dominant, with Subcortical Infarcts and Leukoencephalopathy; CADASIL 125310 J:171887