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Phenotypes Associated with This Genotype
Genotype
MGI:5007812
Allelic
Composition
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0
Genetic
Background
involves: 129 * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat mutation (1 available); any Gt(ROSA)26Sor mutation (392 available)
Notch3Gt(PST033)Byg mutation (0 available); any Notch3 mutation (54 available)
Tg(Tagln-cre)1Her mutation (2 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Vascular smooth muscle cell abnormalities in Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+ Tg(Tagln-cre)1Her/0 and Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Gt(ROSA)26Sor+ Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg Tg(Tagln-cre)1Her/0 mice

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
DO ID OMIM ID(s) Ref(s)
CADASIL 1 DOID:0111035 OMIM:125310
J:171887


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
12/05/2017
MGI 6.11
The Jackson Laboratory