Strain Name:



Cryopreserved - Ready for recovery     Available at the JAX MMRRC

Please refer to the Mutant Mouse Regional Resource Center (MMRRC) for information about 129S-Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat/Mmjax MMRRC Stock Number 033000.
In this ROSA NOTCH3-455 knockin strain, the human NOTCH3 gene is altered by the CADASIL-associated C455R mutation and expression is blocked by an upstream loxP-flanked STOP sequence. Cre-mediated excision results in expression of the mutated NOTCH3 and GFP targeted to the nucleus. Expression in vascular smooth muscle cells is characterized by cell degeneration and the presence of granular osmiophilic deposits. These mice allow inducible expression of a human mutation associated with ischemic stroke.


The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Coisogenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
Generation Definitions
Donating Investigator Spyros Artavanis-Tsakonas,   Harvard Medical School

Homozygotes: Homozygous ROSA NOTCH3-455 mice are viable, fertile, with the CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)-associated C445R mutation inserted into the Gt(ROSA)26Sor locus. Expression of the mutated human NOTCH3 is blocked by an upstream loxP-flanked STOP sequence. When bred to cre expressing mice, the STOP sequence is deleted in the tissues of offspring where Cre recombinase is present, resulting in mutated human NOTCH3 expression and the parallel expression of enhanced green fluorescent protein (GFP) targeted to the nucleus. Expression in vascular smooth muscle cells is characterized by cell degeneration and the presence of granular osmiophilic deposits. These mice allow inducible expression of a human mutation associated with ischemic stroke.

Heterozygote: Not evaluated

A targeting vector was designed with a splice acceptor site, a loxP-flanked PGK neomycin cassette, a human NOTCH3 cDNA encoding a CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy)-associated mutation and an IRES-nuclear EGFP sequence at was inserted into the GT(ROSA)26Sor locus. The CADASIL mutation is an amino acid substitution from cysteine to arginine at amino acid 455 (C455R). The construct was introduced into 129S6/SvEvTac -derived W4 embryonic stem (ES) cells. Correctly targeted ES cells were injected into recipient blastocysts. The resulting "455" chimeric animals were crossed to 129S2/SvEv mice. The mice were crossed to 129S2/SvEv for an unknown number of generations. Upon arrival, mice were bred to 129S1/SvImJ for at least 1 generation to establish the colony.

Control Information

   Wild-type from the colony (approximate)
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Gt(ROSA)26Sor
002292   129-Gt(ROSA)26Sor/J
006053   129-Gt(ROSA)26Sortm1(CAG-EGFP)Luo/J
006067   129-Gt(ROSA)26Sortm2(CAG-Dsred2/EGFP)Luo/J
006041   129-Gt(ROSA)26Sortm3(CAG-EGFP/Dsred2)Luo/J
013205   129S-Gt(ROSA)26Sortm1(NOTCH3)Sat/Mmjax
003310   129S-Gt(ROSA)26Sortm1Sor/J
009043   129S-Gt(ROSA)26Sortm3(CAG-luc)Tyj/J
007844   129S4/SvJae-Gt(ROSA)26Sortm2(FLP*)Sor/J
003946   129S4/SvJaeSor-Gt(ROSA)26Sortm1(FLP1)Dym/J
007689   129S4/SvJaeSor-Gt(ROSA)26Sortm4(attB/attP)Sor/J
017626   B6(Cg)-Gt(ROSA)26Sortm1(CAG-GFP/Eif2c2)Zjh/J
010633   B6(Cg)-Gt(ROSA)26Sortm1(CAG-taulacZ)Bene/J
024540   B6(Cg)-Gt(ROSA)26Sortm1(Sstr3/GFP)Bky/J
008242   B6(Cg)-Gt(ROSA)26Sortm4(Ikbkb)Rsky/J
007676   B6.129(Cg)-Gt(ROSA)26Sortm4(ACTB-tdTomato,-EGFP)Luo/J
006071   B6.129-Gt(ROSA)26Sortm1(CAG-EGFP)Luo/J
007708   B6.129-Gt(ROSA)26Sortm1(HD*103Q)Xwy/J
022793   B6.129-Gt(ROSA)26Sortm1(LRRK2*R1441C)Djmo/J
008463   B6.129-Gt(ROSA)26Sortm1(cre/ERT2)Tyj/J
008606   B6.129-Gt(ROSA)26Sortm1Joe/J
006080   B6.129-Gt(ROSA)26Sortm2(CAG-Dsred2/EGFP)Luo/J
006075   B6.129-Gt(ROSA)26Sortm3(CAG-EGFP/Dsred2)Luo/J
011008   B6.129P2(Cg)-Gt(ROSA)26Sortm1(tTA)Roos/J
017492   B6.129P2-Gt(ROSA)26Sortm1(CAG-Brainbow2.1)Cle/J
024708   B6.129P2-Gt(ROSA)26Sortm1(CAG-RABVgp4,-TVA)Arenk/J
009669   B6.129P2-Gt(ROSA)26Sortm1(DTA)Lky/J
008513   B6.129P2-Gt(ROSA)26Sortm1(Trpv1,ECFP)Mde/J
013586   B6.129P2-Gt(ROSA)26Sortm1Nik/J
013587   B6.129P2-Gt(ROSA)26Sortm3Nik/J
022367   B6.129S4-Gt(ROSA)26Sortm1(CAG-EGFP/Rpl10a,-birA)Wtp/J
009086   B6.129S4-Gt(ROSA)26Sortm1(FLP1)Dym/RainJ
003474   B6.129S4-Gt(ROSA)26Sortm1Sor/J
012930   B6.129S4-Gt(ROSA)26Sortm2(FLP*)Sor/J
009044   B6.129S4-Gt(ROSA)26Sortm3(CAG-luc)Tyj/J
007743   B6.129S4-Gt(ROSA)26Sortm3(phiC31*)Sor/J
009673   B6.129S6(C)-Gt(ROSA)26Sortm3(HIF1A*)Kael/J
002192   B6.129S7-Gt(ROSA)26Sor/J
006148   B6.129X1-Gt(ROSA)26Sortm1(EYFP)Cos/J
017983   B6.Cg-Col1a1tm9(tetO-Dnmt3b_i1)Jae Gt(ROSA)26Sortm1(rtTA*M2)Jae/J
021071   B6.Cg-Gt(ROSA)26Sortm1(CAG-PA-GFP)Rmpl/J
014588   B6.Cg-Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm6(tetO-MSI2)Jae/J
014602   B6.Cg-Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm1(tetO-mCherry)Eggn/J
023749   B6.Cg-Gt(ROSA)26Sortm1(rtTA*M2)Jae Tg(tetO-Pou5f1,-Sox2,-Klf4,-Myc)1Srn/J
006965   B6.Cg-Gt(ROSA)26Sortm1(rtTA*M2)Jae/J
005670   B6.Cg-Gt(ROSA)26Sortm1(rtTA,EGFP)Nagy/J
007914   B6.Cg-Gt(ROSA)26Sortm14(CAG-tdTomato)Hze/J
007920   B6.Cg-Gt(ROSA)26Sortm2(CAG-EYFP)Hze/J
012567   B6.Cg-Gt(ROSA)26Sortm27.1(CAG-COP4*H134R/tdTomato)Hze/J
007903   B6.Cg-Gt(ROSA)26Sortm3(CAG-EYFP)Hze/J
014648   B6.Cg-Gt(ROSA)26Sortm37(H1/tetO-RNAi:Taz)Arte/ZkhuJ
021188   B6.Cg-Gt(ROSA)26Sortm40.1(CAG-aop3/EGFP)Hze/J
007906   B6.Cg-Gt(ROSA)26Sortm6(CAG-ZsGreen1)Hze/J
007909   B6.Cg-Gt(ROSA)26Sortm9(CAG-tdTomato)Hze/J
024750   B6.Cg-Gt(ROSA)26Sortm9(EGFP/Rpl10a)Amc/J
007897   B6.Cg-Tg(Gt(ROSA)26Sor-EGFP)I1Able/J
024179   B6;129-Gt(ROSA)26Sortm1(Actb-T,-GFP)Dalco/J
017455   B6;129-Gt(ROSA)26Sortm1(CAG-COP4*E123T*H134R,-tdTomato)Gfng/J
010527   B6;129-Gt(ROSA)26Sortm1(DTA)Mrc/J
016262   B6;129-Gt(ROSA)26Sortm1(Foxo1/GFP)Jke/J
017962   B6;129-Gt(ROSA)26Sortm1(RAC1*)Jkis/J
008883   B6;129-Gt(ROSA)26Sortm1(SNCA*A53T)Djmo/TmdJ
004847   B6;129-Gt(ROSA)26Sortm1(cre/ERT)Nat/J
021025   B6;129-Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm1(tetO-cre)Haho/J
006911   B6;129-Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm2(tetO-Pou5f1)Jae/J
008516   B6;129-Gt(ROSA)26Sortm1Joe/J
003504   B6;129-Gt(ROSA)26Sortm1Sho/J
021847   B6;129-Gt(ROSA)26Sortm1Ytchn/J
008889   B6;129-Gt(ROSA)26Sortm2(SNCA*119)Djmo/TmdJ
009253   B6;129-Gt(ROSA)26Sortm2Nat/J
004077   B6;129-Gt(ROSA)26Sortm2Sho/J
008886   B6;129-Gt(ROSA)26Sortm3(SNCA*E46K)Djmo/TmdJ
010557   B6;129-Gt(ROSA)26Sortm3(rtTA,tetO-cre/ERT)Nat/J
021039   B6;129-Gt(ROSA)26Sortm5(CAG-Sun1/sfGFP)Nat/J
010523   B6;129P2-Gt(ROSA)26Sortm1(CAG-ALPP)Fawa/J
002073   B6;129S-Gt(ROSA)26Sor/J
018385   B6;129S-Gt(ROSA)26Sortm1(CAG-COX8A/Dendra2)Dcc/J
022516   B6;129S-Gt(ROSA)26Sortm1(Cdkn1c)Jfpa/J
013206   B6;129S-Gt(ROSA)26Sortm1(NOTCH3*R1031C)Sat/Mmjax
018397   B6;129S-Gt(ROSA)26Sortm1.1(CAG-COX8A/Dendra2)Dcc/J
023139   B6;129S-Gt(ROSA)26Sortm1.1Ksvo/J
012569   B6;129S-Gt(ROSA)26Sortm32(CAG-COP4*H134R/EYFP)Hze/J
012570   B6;129S-Gt(ROSA)26Sortm34.1(CAG-Syp/tdTomato)Hze/J
012735   B6;129S-Gt(ROSA)26Sortm35.1(CAG-aop3/GFP)Hze/J
014538   B6;129S-Gt(ROSA)26Sortm38(CAG-GCaMP3)Hze/J
014539   B6;129S-Gt(ROSA)26Sortm39(CAG-hop/EYFP)Hze/J
021875   B6;129S-Gt(ROSA)26Sortm65.1(CAG-tdTomato)Hze/J
021876   B6;129S-Gt(ROSA)26Sortm66.1(CAG-tdTomato)Hze/J
024105   B6;129S-Gt(ROSA)26Sortm95.1(CAG-GCaMP6f)Hze/J
024106   B6;129S-Gt(ROSA)26Sortm96.1(CAG-GCaMP6s)Hze/J
016836   B6;129S4-Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm7(tetO-HIST1H2BJ/GFP)Jae/J
003309   B6;129S4-Gt(ROSA)26Sortm1Sor/J
004598   B6;129S4-Gt(ROSA)26Sortm2Dym/J
007670   B6;129S4-Gt(ROSA)26Sortm3(phiC31*)Sor/J
023035   B6;129S6-Gt(ROSA)26Sortm1(CAG-tdTomato*,-EGFP*)Ees/J
016999   B6;129S6-Gt(ROSA)26Sortm1(xstpx-rtTA2S*M2)Whsu/J
007908   B6;129S6-Gt(ROSA)26Sortm14(CAG-tdTomato)Hze/J
007905   B6;129S6-Gt(ROSA)26Sortm9(CAG-tdTomato)Hze/J
019101   B6N.129S4(B6)-Gt(ROSA)26Sortm1Sor/CjDswJ
016226   B6N.129S4-Gt(ROSA)26Sortm1(FLP1)Dym/J
019013   B6N.129S6(Cg)-Gt(ROSA)26Sortm2(EGFP/cre)Alj/J
023537   B6N.129S6-Gt(ROSA)26Sortm1(CAG-tdTomato*,-EGFP*)Ees/J
019120   BALB/c-Gt(ROSA)26Sortm10(Lmp1)Rsky/J
009670   C.129P2(B6)-Gt(ROSA)26Sortm1(DTA)Lky/J
008603   C.129P2(B6)-Gt(ROSA)26Sortm1(tTA)Roos/J
002955   C.129S7-Gt(ROSA)26Sor/J
007900   C57BL/6-Gt(ROSA)26Sortm1(HBEGF)Awai/J
008517   C57BL/6-Gt(ROSA)26Sortm3(CAG-MIR17-92,-EGFP)Rsky/J
012637   C57BL/6-Gt(ROSA)26Sortm5(Map3k14)Rsky/J
012638   C57BL/6-Gt(ROSA)26Sortm6(Map3k14*)Rsky/J
012343   C57BL/6-Gt(ROSA)26Sortm7(Pik3ca*,EGFP)Rsky/J
012352   C57BL/6-Gt(ROSA)26Sortm8(Map2k1*,EGFP)Rsky/J
012361   C57BL/6-Gt(ROSA)26Sortm9(Rac1*,EGFP)Rsky/J
020458   C57BL/6N-Gt(ROSA)26Sortm13(CAG-MYC,-CD2*)Rsky/J
005420   C;129S7 Gt(ROSA)26Sor-Bmp5cfe-se7J/GrsrJ
008040   CBy.B6-Gt(ROSA)26Sortm1(HBEGF)Awai/J
007898   CBy.Cg-Tg(Gt(ROSA)26Sor-EGFP)I1Able/J
009427   FVB.129S4(B6)-Gt(ROSA)26Sortm1Sor/J
005125   FVB.129S6(B6)-Gt(ROSA)26Sortm1(Luc)Kael/J
016977   FVB.129S6-Gt(ROSA)26Sortm1(Pik3ca*H1047R)Egan/J
006206   FVB.129S6-Gt(ROSA)26Sortm2(HIF1A/luc)Kael/J
012429   FVB.Cg-Gt(ROSA)26Sortm1(CAG-lacZ,-EGFP)Glh/J
010920   FVB;129P2-Gt(ROSA)26Sortm1(birA)Mejr/J
016603   NOD.B6-Gt(ROSA)26Sortm1(HBEGF)Awai/DvsJ
013731   STOCK Gt(ROSA)26Sortm1(CAG-Brainbow2.1)Cle/J
010675   STOCK Gt(ROSA)26Sortm1(CAG-EGFP)Fsh/Mmjax
006331   STOCK Gt(ROSA)26Sortm1(DTA)Jpmb/J
008159   STOCK Gt(ROSA)26Sortm1(Notch1)Dam/J
005130   STOCK Gt(ROSA)26Sortm1(Smo/EYFP)Amc/J
011004   STOCK Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm3(tetO-Pou5f1,-Sox2,-Klf4,-Myc)Jae/J
011011   STOCK Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm4(tetO-Pou5f1,-Sox2,-Klf4,-Myc)Jae/J
011013   STOCK Gt(ROSA)26Sortm1(rtTA*M2)Jae Col1a1tm5(tetO-Pou5f1,-Klf4,-Myc)Jae/J
005572   STOCK Gt(ROSA)26Sortm1(rtTA,EGFP)Nagy/J
008600   STOCK Gt(ROSA)26Sortm1(tTA)Roos/J
018999   STOCK Gt(ROSA)26Sortm1(tTA,tetO-Mir155)Fjsl/J
018998   STOCK Gt(ROSA)26Sortm1(tTA,tetO-Mir21)Fjsl/J
010701   STOCK Gt(ROSA)26Sortm1.1(CAG-EGFP)Fsh/Mmjax
022386   STOCK Gt(ROSA)26Sortm1.1(CAG-EGFP/Rpl10a,-birA)Wtp/J
017596   STOCK Gt(ROSA)26Sortm1.1(rtTA,EGFP)Nagy Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb Tg(tetO-SMN2,-luc)#aAhmb/J
017597   STOCK Gt(ROSA)26Sortm1.1(rtTA,EGFP)Nagy Tg(SMN2)89Ahmb Smn1tm1Msd Tg(SMN2*delta7)4299Ahmb Tg(tetO-SMN2,-luc)#bAhmb/J
010812   STOCK Gt(ROSA)26Sortm1.2(CAG-EGFP)Fsh/Mmjax
017922   STOCK Gt(ROSA)26Sortm10(ACTB-tdTomato)Luo/J
018903   STOCK Gt(ROSA)26Sortm2(EGFP/cre)Alj/J
018906   STOCK Gt(ROSA)26Sortm3(CAG-FLPo/ERT2)Alj/J
013124   STOCK Gt(ROSA)26Sortm3(Gli3)Amc/J
007576   STOCK Gt(ROSA)26Sortm4(ACTB-tdTomato,-EGFP)Luo/J
009674   STOCK Gt(ROSA)26Sortm4(HIF2A*)Kael/J
012266   STOCK Gt(ROSA)26Sortm5(ACTB-tTA)Luo/J
017912   STOCK Gt(ROSA)26Sortm6(ACTB-EGFP*,-tdTomato)Luo/J
013123   STOCK Gt(ROSA)26Sortm6(Gli1)Amc/J
017921   STOCK Gt(ROSA)26Sortm7(ACTB-EGFP*)Luo/J
017909   STOCK Gt(ROSA)26Sortm8(ACTB-EGFP*,-tTA2)Luo/J
007577   STOCK Tg(Gt(ROSA)26Sor-BCHE*G117H)837Loc/J
007896   STOCK Tg(Gt(ROSA)26Sor-EGFP)I1Able/J
View Strains carrying other alleles of Gt(ROSA)26Sor     (153 strains)

Additional Web Information

Information about the Rosa26 locus on the Soriano lab web page


Phenotype Information

View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Cardiovascular Research
Ischemia studies

Research Tools
Cardiovascular Research
      Cre-lox System
Fluorescent Proteins

Genes & Alleles

Gene & Allele Information provided by MGI

Allele Symbol Gt(ROSA)26Sortm2(NOTCH3*C455R)Sat
Allele Name targeted mutation 2, Spyros Artavanis-Tsakonas
Allele Type Targeted (Floxed/Frt)
Common Name(s) NOTCH3C455R;
Strain of Origin129S6/SvEvTac
Gene Symbol and Name Gt(ROSA)26Sor, gene trap ROSA 26, Philippe Soriano
Chromosome 6
Gene Common Name(s) AV258896; Gtrgeo26; Gtrosa26; R26; ROSA26; beta geo; expressed sequence AV258896; gene trap ROSA 26; gene trap ROSA b-geo 26;
Molecular Note A targeting vector containing a splice acceptor site, a floxed PGK neomycin cassette, a human NOTCH3 cDNA with a C455R mutation and an IRES-nuclear GFP sequence was inserted via homologous recombination. [MGI Ref ID J:171887]


Genotyping Information

Genotyping Protocols

ROSA26 Generic GFP, Melt Curve Analysis

Helpful Links

Genotyping resources and troubleshooting


References provided by MGI

Selected Reference(s)

Arboleda-Velasquez JF; Manent J; Lee JH; Tikka S; Ospina C; Vanderburg CR; Frosch MP; Rodriguez-Falcon M; Villen J; Gygi S; Lopera F; Kalimo H; Moskowitz MA; Ayata C; Louvi A; Artavanis-Tsakonas S. 2011. PNAS Plus: Hypomorphic Notch 3 alleles link Notch signaling to ischemic cerebral small-vessel disease. Proc Natl Acad Sci U S A 108(21):E128-35. [PubMed: 21555590]  [MGI Ref ID J:171887]

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryWhile maintaining a live colony, these mice are bred as homozygotes.

Pricing and Purchasing

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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.

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