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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat
targeted mutation 3, Spyros Artavanis-Tsakonas
MGI:5007799
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cn1
 		Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Notch3Gt(PST033)Byg/Notch3Gt(PST033)Byg
Tg(Tagln-cre)1Her/0 		involves: 129 * C57BL/6 * SJL MGI:5007810
cn2
 		Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Tg(Tagln-cre)1Her/0 		involves: 129S2/SvPas * 129S6/SvEvTac * C57BL/6 * SJL MGI:5007819


Genotype
MGI:5007810
cn1
Allelic
Composition		 Gt(ROSA)26Sortm3(NOTCH3*R1031C)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)26Sortm3(NOTCH3*R1031C)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
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 at 1 year of age but is rescued at 3 to 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 at 1 year of age but is rescued at 3 to 6 months of age

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
CADASIL 1 DOID:0111035 OMIM:125310
 J:171887




Genotype
MGI:5007819
cn2
Allelic
Composition		 Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat/Gt(ROSA)26Sor+
Tg(Tagln-cre)1Her/0
Genetic
Background		 involves: 129S2/SvPas * 129S6/SvEvTac * C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sortm3(NOTCH3*R1031C)Sat mutation (1 available); any Gt(ROSA)26Sor mutation (942 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 aorta morphology ( J:171887 )
• thinning of the vascular smooth muscle layers
abnormal brain vasculature morphology ( J:171887 )
• osmiophilic granular deposits in mice older than 12 months of age
abnormal vascular smooth muscle morphology ( J:171887 )
• vascular smooth muscle cell abnormalities with intracellular inclusions and lipid droplets that get more severe with age
• thinning of the vascular smooth muscle layers

muscle
abnormal vascular smooth muscle morphology ( J:171887 )
• vascular smooth muscle cell abnormalities with intracellular inclusions and lipid droplets that get more severe with age
• thinning of the vascular smooth muscle layers

nervous system
abnormal brain vasculature morphology ( J:171887 )
• osmiophilic granular deposits in mice older than 12 months of age

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