All Phenotypes Notch3<+> MGI Mouse

Phenotypes associated with this allele

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	Notch3^em1Ecan/Notch3⁺	C57BL/6J-Notch3^em1Ecan	MGI:6400224
ht2	Notch3^{tm1.1(cre/ERT2)Sat}/Notch3⁺	C57BL/6-Notch3^{tm1.1(cre/ERT2)Sat}	MGI:5304926
ht3	Notch3^tm1.1Dwr/Notch3⁺	involves: 129S/SvEv * Swiss	MGI:5462096
ht4	Notch3^tm1Ul/Notch3⁺	Not Specified	MGI:3528982
cn5	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Notch3^{Gt(PST033)Byg}/Notch3⁺ Tg(Mx1-cre)1Cgn/0	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * CBA	MGI:5009045
cn6	Gt(ROSA)26Sor^{tm2(CAG-Dsred2/EGFP)Luo}/Gt(ROSA)26Sor^{tm1(Notch3*)Sfr} Notch3^{tm1.1(cre/ERT2)Sat}/Notch3⁺	involves: 129S1/Sv * 129X1/SvJ * C57BL/6NTac	MGI:5556107

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

growth/size/body

decreased body weight ( J:270682 )

• weight is about 10% lower than in controls at 1 month of age and this difference in weight becomes less pronounced with age, with 4-month-old mice weighing about 5% less than controls

skeleton

abnormal osteoclast differentiation ( J:270682 )

• bone-marrow derived macrophage cultures show increased osteoclast formation

increased osteoblast proliferation ( J:270682 )

• calvarial osteoblasts grown in culture exhibit a 1.5 higher proliferation rate than wild-type cells

increased osteoclast cell number ( J:270682 )

• osteoclast surface/bone surface is increased in males at 1 month of age

• however, osteoclast number/bone perimeter is not affected and osteoclast surface and osteoclast number are not affected in 4-month-old male femurs

decreased bone mineral density ( J:270682 )

• 1-month-old mice exhibit cancellous and cortical bone osteopenia

• during maturation, cancellous and cortical bones are restored in females but not in males, which exhibit cancellous bone osteopenia at 4 months of age

decreased bone volume ( J:270682 )

• femora of males and females show decreased bone volume/tissue volume at 1 month of age

• at 4 months of age, males show decreased bone volume/tissue volume in femurs

decreased trabecular bone volume ( J:270682 )

• 35-60% decrease in cancellous bone volume in 1-month-old mice

• at 4 months of age, males, but not females, continue to show a decrease in cancellous bone volume of about 30%

decreased compact bone thickness ( J:270682 )

• cortical bone is thin and porous in 1-month old mice, however total bone area is not different

• however, by 4 months of age, cortical bone is not altered

increased osteoblast cell number ( J:270682 )

• approximate 2-fold increase in the number of osteoblasts and in osteoblast surface/bone surface in femurs at 1-month of age

• at 4 months of age, males show a 60% increase in osteoblast number in femurs

increased osteocyte number ( J:270682 )

• 13-33% increase in osteocyte cell density in femurs at 1-month of age

abnormal bone trabecula morphology ( J:270682 )

• mice show a predominance of rodlike trabeculae

• however, trabecular thickness is normal

decreased bone trabecula number ( J:270682 )

• in males and females at 1-month and 4-months of age

decreased trabecular bone connectivity density ( J:270682 )

abnormal bone mineralization ( J:270682 )

• increase in mineral apposition rate in males at 1 month of age, with a nonsignificant increase in bone formation rate

increased bone ossification ( J:270682 )

• at 4 months of age, males show elevated bone formation rate

hematopoietic system

abnormal osteoclast differentiation ( J:270682 )

• bone-marrow derived macrophage cultures show increased osteoclast formation

increased osteoclast cell number ( J:270682 )

• osteoclast surface/bone surface is increased in males at 1 month of age

• however, osteoclast number/bone perimeter is not affected and osteoclast surface and osteoclast number are not affected in 4-month-old male femurs

immune system

abnormal osteoclast differentiation ( J:270682 )

• bone-marrow derived macrophage cultures show increased osteoclast formation

increased osteoclast cell number ( J:270682 )

• osteoclast surface/bone surface is increased in males at 1 month of age

• however, osteoclast number/bone perimeter is not affected and osteoclast surface and osteoclast number are not affected in 4-month-old male femurs

cellular

abnormal osteoclast differentiation ( J:270682 )

• bone-marrow derived macrophage cultures show increased osteoclast formation

increased osteoblast proliferation ( J:270682 )

• calvarial osteoblasts grown in culture exhibit a 1.5 higher proliferation rate than wild-type cells

behavior/neurological

behavior/neurological phenotype ( J:270682 )

N	• mice exhibit no obvious neurological alternations

Allelic Composition	Notch3^{tm1.1(cre/ERT2)Sat}/Notch3⁺
Genetic Background	C57BL/6-Notch3^{tm1.1(cre/ERT2)Sat}

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch3^{tm1.1(cre/ERT2)Sat} mutation (0 available); any Notch3 mutation (95 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

normal phenotype

no abnormal phenotype detected ( J:179632 )

• heterozygotes are viable and fertile

Allelic Composition	Notch3^tm1.1Dwr/Notch3⁺
Genetic Background	involves: 129S/SvEv * Swiss

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch3^tm1.1Dwr mutation (0 available); any Notch3 mutation (95 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

behavior/neurological

decreased exploration in new environment ( J:191454 )

• somewhat

abnormal motor capabilities/coordination/movement ( J:191454 )

• in 9 of 73 mice develop overt permanent motor disability, ataxia or both

ataxia ( J:191454 )

• in 9 of 73 mice, 3 of which show staggering ataxic gait

paresis ( J:191454 )

• of one or more limb in 8 of 9 mice with motor disabilities

cardiovascular system

cardiovascular system phenotype ( J:191454 )

N	• mice exhibit normal vascular smooth muscle cell remodeling or growth and normal cerebral capillary densities

abnormal artery morphology ( J:191454 )

• mice exhibit arteriopathy with granular osminophilic material deposits in the basal lamina of the arterial smooth muscle cells and surrounding matrix in brain and tail arteries

nervous system

gliosis ( J:191454 )

• in some mice

brain lesion ( J:191454 )

• micro-bleeds, hemosiderin deposits or hemosiderin-containing macrophages, perivascular inflammatory infiltration, gliosis, thrombosis, perivascular fibrin(ogen) deposition, microinfarctions characterized by foci with pallor and cell loss around small cystic cavities, and enlargement of the Virchow-Robin spaces creating perivascular lacunae

homeostasis/metabolism

abnormal brain thrombosis ( J:191454 )

• in the brain vasculature of some mice

cellular

gliosis ( J:191454 )

• in some mice

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
CADASIL 1		DOID:0111035	OMIM:125310	J:191454

Allelic Composition	Notch3^tm1Ul/Notch3⁺
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch3^tm1Ul mutation (0 available); any Notch3 mutation (95 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

cardiovascular system

cardiovascular system phenotype ( J:95927 )

N	• vascular smooth muscle is normal in the thoracic aorta and common carotid arteries and electron microscopic examination detected no abnormalities in the thoracic and abdominal aortas or the carotid, leptomeningeal, and cerebral cortical penetrating arteries

normal phenotype

no abnormal phenotype detected ( J:95927 )

• heterozygotes are viable with normal growth, reproduction, behavior, and morphology of the brain, heart, lung, eye, and skin

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
NOT	CADASIL 1	DOID:0111035	OMIM:125310	J:95927

Allelic Composition	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Notch3^{Gt(PST033)Byg}/Notch3⁺ Tg(Mx1-cre)1Cgn/0
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (116 available)
Notch2^tm1Rko mutation (0 available); any Notch2 mutation (97 available)
Notch3^{Gt(PST033)Byg} mutation (0 available); any Notch3 mutation (95 available)
Tg(Mx1-cre)1Cgn mutation (10 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

hematopoietic system

extramedullary hematopoiesis ( J:172442 )

increased hematopoietic cell number ( J:172442 )

• myeloproliferative disease

abnormal spleen morphology ( J:172442 )

• massive invasion of myeloid cells

enlarged spleen ( J:172442 )

increased spleen red pulp amount ( J:172442 )

immune system

abnormal spleen morphology ( J:172442 )

• massive invasion of myeloid cells

enlarged spleen ( J:172442 )

increased spleen red pulp amount ( J:172442 )

growth/size/body

enlarged spleen ( J:172442 )

Allelic Composition	Gt(ROSA)26Sor^{tm2(CAG-Dsred2/EGFP)Luo}/Gt(ROSA)26Sor^{tm1(Notch3*)Sfr} Notch3^{tm1.1(cre/ERT2)Sat}/Notch3⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ * C57BL/6NTac

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Gt(ROSA)26Sor^{tm1(Notch3*)Sfr} mutation (0 available); any Gt(ROSA)26Sor mutation (1095 available)
Gt(ROSA)26Sor^{tm2(CAG-Dsred2/EGFP)Luo} mutation (2 available); any Gt(ROSA)26Sor mutation (1095 available)
Notch3^{tm1.1(cre/ERT2)Sat} mutation (0 available); any Notch3 mutation (95 available)

♀	phenotype observed in females
♂	phenotype observed in males
N	normal phenotype

endocrine/exocrine glands

decreased mammary gland epithelial cell proliferation ( J:201985 )

• following 4 weeks of tamoxifen treatment clonal expansion of cre expressing cells is markedly reduced

cellular

decreased mammary gland epithelial cell proliferation ( J:201985 )

• following 4 weeks of tamoxifen treatment clonal expansion of cre expressing cells is markedly reduced

integument

decreased mammary gland epithelial cell proliferation ( J:201985 )

• following 4 weeks of tamoxifen treatment clonal expansion of cre expressing cells is markedly reduced

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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