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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat
targeted mutation 2, Spyros Artavanis-Tsakonas
MGI:5007800
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
cn1
 		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 MGI:5007812


Genotype
MGI:5007812
cn1
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 (942 available)
Notch3Gt(PST033)Byg mutation (0 available); any Notch3 mutation (93 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
abnormal vascular smooth muscle morphology ( J:171887 )
• vascular smooth muscle cell abnormalities with intracellular inclusions are seen at 6 months of age

homeostasis/metabolism
increased susceptibility to ischemic brain injury ( J:171887 )
• increased susceptibility to ischemic injury seen in Notch3 null mice is not rescued by expression of the mutant human NOTCH3

muscle
abnormal vascular smooth muscle morphology ( J:171887 )
• vascular smooth muscle cell abnormalities with intracellular inclusions are seen at 6 months of age

nervous system
increased susceptibility to ischemic brain injury ( J:171887 )
• 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 Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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Send questions and comments to User Support. 		last database update
04/09/2024
MGI 6.23 		The Jackson Laboratory