All Phenotypes Notch1<tm2Rko> MGI Mouse

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Phenotypes associated with this allele

Allele Symbol
Allele Name
Allele ID

Notch1^tm2Rko
targeted mutation 2, Raphael Kopan
MGI:3044585

Summary

12 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cn1	Notch1^tm2Rko/Notch1^tm3(cre)Rko	involves: 129X1/SvJ * C57BL/6	MGI:5009691
cn2	Notch1^tm2Rko/Notch1^tm4(cre)Rko	involves: 129X1/SvJ * C57BL/6	MGI:5009540
cn3	Notch1^tm2Rko/Notch1^tm2Rko Tg(KRT14-rtTA)F42Efu/0 Tg(tetO-cre)1Jaw/0	involves: 129 * 129X1/SvJ * C57BL/6 * FVB/N	MGI:5512989
cn4	Notch1^tm1Grid/Notch1^tm2Rko Foxg1^tm1(cre)Skm/Foxg1⁺	involves: 129 * C57BL/6J * Swiss Webster	MGI:3776429
cn5	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Notch3^{Gt(PST033)Byg}/Notch3^{Gt(PST033)Byg} Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd * 129X1/SvJ	MGI:3525189
cn6	Notch1^tm2Rko/Notch1^tm2Rko Notch3^{Gt(PST033)Byg}/Notch3^{Gt(PST033)Byg} Tg(Msx2-cre)5Rem/0	involves: 129P2/OlaHsd * 129X1/SvJ	MGI:3525186
cn7	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Notch3^{Gt(PST033)Byg}/Notch3^{Gt(PST033)Byg} Tg(Mx1-cre)1Cgn/0	involves: 129P2/OlaHsd * 129X1/SvJ * C57BL/6 * CBA	MGI:5009039
cn8	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
cn9	Notch1^tm2Rko/Notch1^tm2Rko H2az2^{Tg(Wnt1-cre)11Rth}/H2az2⁺	involves: 129X1/SvJ * C57BL/6 * CBA	MGI:5318530
cn10	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Tg(Msx2-cre)5Rem/0	involves: 129X1/SvJ * C57BL/6 * CBA	MGI:3525181
cn11	Notch1^tm2Rko/Notch1^tm2Rko Tg(Nes-cre)1Kln/0	involves: 129X1/SvJ * C57BL/6J * SJL	MGI:3044600
cn12	Notch1^tm2Rko/Notch1^tm2Rko Tg(Msx2-cre)5Rem/0	Not Specified	MGI:3525169

Allelic Composition	Notch1^tm2Rko/Notch1^tm3(cre)Rko
Genetic Background	involves: 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)
Notch1^tm3(cre)Rko mutation (1 available); any Notch1 mutation (115 available)

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

Corneal plaques and increased angiogenesis in the ear of Notch1^tm2Rko/Notch1^tm3(cre)Rko mice

growth/size/body

enlarged spleen ( J:171829 )

• reactive splenomegaly is observed at post mortem

mortality/aging

premature death ( J:171829 )

• life expectancy is significantly reduced in mutants (420 days) compared to wild-type (600 days)

neoplasm

increased hemangioma incidence ( J:171829 )

• 11/13 mice have hemangiosarcoma/vascular tumors, some of which involve multiple organs including the ovary, testis, skin, lymph node, uterus, and colon; liver is the primary site (82% of mice)

vision/eye

abnormal cornea morphology ( J:171829 )

• corneal hyperplasia develops by 2-3 months in all animals

corneal vascularization ( J:171829 )

• normal cornea is replaced by a vascularized epidermis-like structure

corneal deposits ( J:171829 )

• mice develop corneal plaques by 2-3 months; this increases in severity with age

hematopoietic system

extramedullary hematopoiesis ( J:171829 )

• observed in the spleen in granulocytic, erythrocytic, and megakaryocytic lineages with no bias in B/T cell lineages

abnormal spleen morphology ( J:171829 )

• marked expansion of the interfollicular compartments is observed

enlarged spleen ( J:171829 )

• reactive splenomegaly is observed at post mortem

liver/biliary system

chronic liver inflammation ( J:171829 )

• dense, chronic inflammatory infiltrates are observed around the bile ducts

abnormal liver morphology ( J:171829 )

• liver contains numerous reddened large blood filled spaces consistent with occurrence of vascular tumors

abnormal hepatic cord morphology ( J:171829 )

• hepatic cords are separated by inflammatory infiltrate

abnormal liver parenchyma morphology ( J:171829 )

• normal parenchyma is replaced either by solid, hypercellular tissue composed of spindle and epithelioid cells interspersed with many vascular channels or by irregular vascular channels lined by cells with scant-to-moderate eosinophilic cytoplasm and round-to-oval nuclei; nuclei of these cells of endothelial origin were hyperchromatic or contained coarse chromatin

abnormal liver lobule morphology ( J:171829 )

• liver lobes have irregular contours

dilated liver sinusoidal space ( J:171829 )

pale liver ( J:171829 )

• on post mortem liver is observed to be pale, with many irregular reddened foci; some patches are almost white

cardiovascular system

dilated liver sinusoidal space ( J:171829 )

increased angiogenesis ( J:171829 )

• increased angiogenesis is observed in the ear

hemoperitoneum ( J:171829 )

• observed in 6/7 naturally deceased mice

corneal vascularization ( J:171829 )

• normal cornea is replaced by a vascularized epidermis-like structure

immune system

abnormal spleen morphology ( J:171829 )

• marked expansion of the interfollicular compartments is observed

enlarged spleen ( J:171829 )

• reactive splenomegaly is observed at post mortem

chronic liver inflammation ( J:171829 )

• dense, chronic inflammatory infiltrates are observed around the bile ducts

Allelic Composition	Notch1^tm2Rko/Notch1^tm4(cre)Rko
Genetic Background	involves: 129X1/SvJ * C57BL/6

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)
Notch1^tm4(cre)Rko mutation (1 available); any Notch1 mutation (115 available)

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

Embryonic lethality in Notch1^tm2Rko/Notch1^tm4(cre)Rko embryos due to the failure of angiogenic remodeling

embryo

abnormal vitelline vasculature morphology ( J:171829 )

• defective angiogenic remodeling of the yolk sac vasculature is observed

mortality/aging

embryonic lethality during organogenesis, complete penetrance ( J:171829 )

• death at E10.5 is observed, likely resulting from higher loss of heterozygosity (LOH) frequency compared to that in Notch1^tm2Rko/Notch1^tm3(cre)Rko embryos; higher LOH occurs because of improved cre expression

cardiovascular system

abnormal vitelline vasculature morphology ( J:171829 )

• defective angiogenic remodeling of the yolk sac vasculature is observed

Allelic Composition	Notch1^tm2Rko/Notch1^tm2Rko Tg(KRT14-rtTA)F42Efu/0 Tg(tetO-cre)1Jaw/0
Genetic Background	involves: 129 * 129X1/SvJ * C57BL/6 * FVB/N

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)
Tg(KRT14-rtTA)F42Efu mutation (1 available)
Tg(tetO-cre)1Jaw mutation (5 available)

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

vision/eye

Meibomian gland degeneration ( J:194073 )

• mutants treated with doxycycline from P1 to P16 show degeneration of ocular glands, including lacrimal and Meibomian glands

lacrimal gland degeneration ( J:194073 )

• mutants treated with doxycycline from P1 to P16 show degeneration of ocular glands, including lacrimal and Meibomian glands

decreased conjunctiva goblet cell number ( J:194073 )

• mutants treated with doxycycline from P1 to P16 exhibit loss of conjunctival goblet cells

dry eyes ( J:194073 )

• mutants treated with doxycycline from P1 to P16 have poor tear volume

endocrine/exocrine glands

Meibomian gland degeneration ( J:194073 )

• mutants treated with doxycycline from P1 to P16 show degeneration of ocular glands, including lacrimal and Meibomian glands

lacrimal gland degeneration ( J:194073 )

• mutants treated with doxycycline from P1 to P16 show degeneration of ocular glands, including lacrimal and Meibomian glands

integument

Meibomian gland degeneration ( J:194073 )

• mutants treated with doxycycline from P1 to P16 show degeneration of ocular glands, including lacrimal and Meibomian glands

alopecia ( J:194073 )

• mutants treated with doxycycline from P1 to P16 show hair loss on the skin and eyelids

Allelic Composition	Notch1^tm1Grid/Notch1^tm2Rko Foxg1^tm1(cre)Skm/Foxg1⁺
Genetic Background	involves: 129 * C57BL/6J * Swiss Webster

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Foxg1^tm1(cre)Skm mutation (2 available); any Foxg1 mutation (28 available)
Notch1^tm1Grid mutation (0 available); any Notch1 mutation (115 available)
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)

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

hearing/vestibular/ear

increased cochlear inner hair cell number ( J:132241 )

• increases in inner hair cell number are more than in Dll1^tm1Gos/Dll1^tm2Gos Jag2^tm1Grid/Jag2^tm1Grid mice

decreased organ of Corti supporting cell number ( J:132241 )

• decreases in inner hair cell number are slightly than in Dll1^tm1Gos/Dll1^tm2Gos Jag2^tm1Grid/Jag2^tm1Grid mice or Dll1^tm1Gos Jag2^tm1Grid homozygotes

nervous system

increased cochlear inner hair cell number ( J:132241 )

• increases in inner hair cell number are more than in Dll1^tm1Gos/Dll1^tm2Gos Jag2^tm1Grid/Jag2^tm1Grid mice

Allelic Composition	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Notch3^{Gt(PST033)Byg}/Notch3^{Gt(PST033)Byg} Tg(Msx2-cre)5Rem/0
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)
Notch2^tm1Rko mutation (0 available); any Notch2 mutation (97 available)
Notch3^{Gt(PST033)Byg} mutation (0 available); any Notch3 mutation (93 available)
Tg(Msx2-cre)5Rem mutation (2 available)

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

endocrine/exocrine glands

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

integument

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

thick epidermis ( J:94517 )

• had frequent acanthotic and perakeratotic scales

scaly skin ( J:94517 )

• had frequent perakeratotic scales

Allelic Composition	Notch1^tm2Rko/Notch1^tm2Rko Notch3^{Gt(PST033)Byg}/Notch3^{Gt(PST033)Byg} Tg(Msx2-cre)5Rem/0
Genetic Background	involves: 129P2/OlaHsd * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)
Notch3^{Gt(PST033)Byg} mutation (0 available); any Notch3 mutation (93 available)
Tg(Msx2-cre)5Rem mutation (2 available)

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

integument

abnormal hair follicle morphology ( J:94517 )

• displayed similar follicular morphologies as mutant mice homozygous for Notch1^tm1Shn expressing the Tg(Msx2-cre)5Rem transgene

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

Find Mice

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

hematopoietic system

abnormal definitive hematopoiesis ( J:172442 )

• increase in the granulocyte/monocyte progenitors cell population

extramedullary hematopoiesis ( J:172442 )

increased hematopoietic cell number ( J:172442 )

• increase in the granulocyte/monocyte progenitors cell population

abnormal spleen morphology ( J:172442 )

• massive invasion of myeloid cells

enlarged spleen ( J:172442 )

increased spleen red pulp amount ( J:172442 )

neoplasm

increased leukemia incidence ( J:172442 )

• similarity to chronic myelomonocytic leukemia

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	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

♀	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	Notch1^tm2Rko/Notch1^tm2Rko H2az2^{Tg(Wnt1-cre)11Rth}/H2az2⁺
Genetic Background	involves: 129X1/SvJ * C57BL/6 * CBA

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
H2az2^{Tg(Wnt1-cre)11Rth} mutation (2 available); any H2az2 mutation (26 available)
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)

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

mortality/aging

perinatal lethality, complete penetrance ( J:181120 )

• mutant mice are not viable and die at birth

craniofacial

craniofacial phenotype ( J:181120 )

N	• normal palate formation

Allelic Composition	Notch1^tm2Rko/Notch1^tm2Rko Notch2^tm1Rko/Notch2^tm1Rko Tg(Msx2-cre)5Rem/0
Genetic Background	involves: 129X1/SvJ * C57BL/6 * CBA

Find Mice

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

mortality/aging

premature death ( J:94517 )

• die around P25

endocrine/exocrine glands

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

integument

absent sebaceous gland ( J:94517 )

• loss of sebaceous glands in embryo-deleted follicles

progressive hair loss ( J:94517 )

• regions of bald skin (where Cre was expressed in E9.5 developing follicles) alternating with hair (where Cre was expressed at P1 in follicle cells), however this hair was progressively lost as mice age

absent hair follicle inner root sheath ( J:94517 )

• embryo-deleted follicles contain a melanin-producing core with large, loosely packed cells replacing the inner root sheath

hair follicle degeneration ( J:94517 )

• by P25, P1-deleted follicles are degenerating instead of being in telogen

epidermal hyperplasia ( J:94517 )

scaly skin ( J:94517 )

• regions of scaly skin

Allelic Composition	Notch1^tm2Rko/Notch1^tm2Rko Tg(Nes-cre)1Kln/0
Genetic Background	involves: 129X1/SvJ * C57BL/6J * SJL

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)
Tg(Nes-cre)1Kln mutation (4 available)

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

cellular

abnormal apoptosis ( J:90392 )

• at E10 the total number of apoptotic cells and relative percentage of apoptotic cells to progenitor cells in the forebrain-midbrain junction is significantly reduced compared to littermate controls

nervous system

increased neuron number ( J:90392 )

• the number of postmitotic neurons is increased in the telencephalon at E11.5 - 12.5 compared to littermate controls

Allelic Composition	Notch1^tm2Rko/Notch1^tm2Rko Tg(Msx2-cre)5Rem/0
Genetic Background	Not Specified

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Notch1^tm2Rko mutation (3 available); any Notch1 mutation (115 available)
Tg(Msx2-cre)5Rem mutation (2 available)

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

endocrine/exocrine glands

small sebaceous gland ( J:94517 )

• severely reduced sebaceous glands in embryo-deleted skin

integument

small sebaceous gland ( J:94517 )

• severely reduced sebaceous glands in embryo-deleted skin

abnormal hair growth ( J:94517 )

• mosaic pattern of hair growth (regions of finer, shorter, and wavy hairs that showed twisted, knotted morphology resulting from Cre expression at E9.5 and regions of normal hairs resulting from Cre expression at P1) that was maintained throughout life

short hair ( J:94517 )

waved hair ( J:94517 )

• regions of wavy hair that have a twisted, knotted morphology

abnormal hair shaft morphology ( J:94517 )

• bulbous perturbations in the shaft

abnormal hair follicle morphology ( J:94517 )

• medulla layer of embryo-deleted follicles was disorganized, with two smaller cells, instead of one, packed inside the medulla with fewer, disorganized trichohyalin granules

disorganized inner root sheath cells ( J:94517 )

• the inner root sheath Henle's layer of embryo-deleted follicles is improperly organized with up to four cell tips in the mutant hair instead of two

• however, the cortex is normal

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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