Strain Name:

B6;129S1-Notch3tm1Grid/J

Stock Number:

010547

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Availability:

Repository- Live

These Notch3d1 mutant mice exhibit abnormal arterial wall morphology. This mutant mouse strain may be useful in studies of the Notch pathway during development, vascular development and angiogenesis.

Description

Strain Information

Type Mutant Stock; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Mating SystemHomozygote x Homozygote         (Female x Male)   11-DEC-12
Specieslaboratory mouse
GenerationF?+F7 (11-DEC-13)
Generation Definitions
 
Donating Investigator Tom Gridley,   Maine Medical Center Research Institute

Description
Mice that are homozygous for the targeted mutation are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No gene product (mRNA or protein) is detected by Northern blot analysis of brain, lung and heart tissue or Western blot analysis of lung and brain tissue from homozygotes. Homozygotes exhibit disorganized artery wall morphology, and thin vascular smooth muscle cell coat and processes.

Development
A targeting vector containing a PGK-neo cassette was used to delete 2.5kb of sequence encoding the EGF repeats 8 through 12. The construct was electroporated into 129S1/Sv-Oca2+ Tyr+ Kitl+ derived CJ7 embryonic stem (ES) cells. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The resulting male chimeric animals were crossed to C57BL/6J females, and then C57BL/6J for 3 generations. The mice were bred to C57BL/6J for one generation to establish the distribution colony.

Control Information

  Control
   101043 B6129SF1/J (approximate)
   101045 B6129SF2/J (approximate)
 
  Considerations for Choosing Controls

Related Strains

Facebase: models
007664   129S-Efnb1tm1Sor/J
000646   A/J
000647   A/WySnJ
005709   B6.129-Skitm1Cco/J
002619   B6.129-Tgfb3tm1Doe/J
007453   B6.129P2(Cg)-Dhcr7tm1Gst/J
010525   B6.129S-Notch2tm3Grid/J
010616   B6.129S1-Jag1tm1Grid/J
010546   B6.129S1-Jag2tm1Grid/J
010620   B6.129S1-Notch2tm1Grid/J
009387   B6.129S1-Osr1tm1Jian/J
009386   B6.129S1-Osr2tm1Jian/J
010621   B6.129S1-Snai1tm2.1Grid/J
010617   B6.129S1-Snai2tm1Grid/J
003865   B6.129S2-Itgavtm1Hyn/J
003755   B6.129S4-Meox2tm1(cre)Sor/J
016902   B6.129S5-Irf6Gt(OST398253)Lex/J
003336   B6.129S7-Cdkn1ctm1Sje/J
012843   B6.129X1(Cg)-Slc32a1tm1.1Bgc/J
000026   B6.C3-Gli3Xt-J/J
004275   B6.Cg-Fignfi/Frk
012844   B6.Cg-Gad1tm1.1Bgc/J
006382   B6;129-Casktm1Sud/J
002711   B6;129-Gabrb3tm1Geh/J
004293   B6;129-Shhtm2Amc/J
012603   B6;129-Tgfbr2tm1Karl/J
010618   B6;129S-Jag1tm2Grid/J
010686   B6;129S-Snai1tm2Grid/J
009389   B6;129S1-Bambitm1Jian/J
010619   B6;129S1-Lfngtm1Grid/J
010544   B6;129S1-Notch4tm1Grid/J
010722   B6;129S1-Snai2tm2Grid/J
012463   B6;129S4-Foxd1tm1(GFP/cre)Amc/J
022358   B6;129S6-Rr23tm1Axvi/Mmjax
022359   B6;129S6-Rr24tm1Axvi/Mmjax
022360   B6;129S6-Rr25tm1Axvi/Mmjax
003277   B6;129S7-Acvr2atm1Zuk/J
002788   B6;129S7-Fsttm1Zuk/J
002990   B6;129S7-Inhbatm1Zuk/J
000523   B6By.Cg-Eh/J
000278   B6C3Fe a/a-Papss2bm Hps1ep Hps6ru/J
000515   B6CBACa Aw-J/A-SfnEr/J
001434   C3HeB/FeJ x STX/Le-Mc1rE-so Gli3Xt-J Zeb1Tw/J
000252   DC/LeJ
005057   FVB.129-Kcnj2tm1Swz/J
012655   FVB.A-Irf6clft1/BeiJ
013100   FVB.C-Prdm16csp1/J
017437   FVB/N-Ckap5TgTn(sb-cHS4,Tyr)2320F-1Ove/J
017438   FVB/N-MidnTg(Tyr)2261EOve/J
017609   FVB/N-Rr16Tn(sb-Tyr)1HCebOve/J
017598   FVB/N-Sdccag8Tn(sb-Tyr)2161B.CA1C2Ove/J
017608   FVB/N-Skor2Tn(sb-Tyr)1799B.CA7BOve/J
017436   FVB/N-Tapt1TgTn(sb-cHS4,Tyr)2508GOve/J
016870   FVB/NJ-Ap2b1Tg(Tyr)427Ove/EtevJ
017434   FVB;B6-Cramp1lTgTn(sb-rtTA,Tyr)2447AOve/J
017594   FVB;B6-Eya4TgTn(Prm1-sb10,sb-Tyr)1739AOve/J
017435   FVB;B6-SlmapTn(sb-rtTA)2426B.SB4Ove/J
003318   STOCK Shhtm1Amc/J
003102   STOCK Tgfb2tm1Doe/J
018624   STOCK Tgfb3tm2(Tgfb1)Vk/J
008469   STOCK Wnt9btm1.2Amc/J
View Facebase: models     (61 strains)

Strains carrying   Notch3tm1Grid allele
023807   FVB.129S1(B6)-Notch3tm1Grid/MshaJ
View Strains carrying   Notch3tm1Grid     (1 strain)

Strains carrying other alleles of Notch3
005330   C57BL/6J-Notch3hpbk/GrsrJ
View Strains carrying other alleles of Notch3     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Cerebral Arteriopathy, Autosomal Dominant, with Subcortical Infarcts and Leukoencephalopathy; CADASIL   (NOTCH3)
Myofibromatosis, Infantile, 2; IMF2   (NOTCH3)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Notch3tm1Grid/Notch3tm1Grid

        involves: 129S1/Sv * C57BL/6J
  • normal phenotype
  • no abnormal phenotype detected
    • mice were viable and fertile   (MGI Ref ID J:87272)

The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain.

Notch3tm1Grid/Notch3tm1Grid

        129S1/SvImJ-Notch3tm1Grid
  • normal phenotype
  • no abnormal phenotype detected
    • mice were viable and fertile   (MGI Ref ID J:87272)

Notch3tm1Grid/Notch3tm1Grid

        involves: 129S1/Sv
  • cardiovascular system phenotype
  • abnormal artery morphology
    • enlarged arteries with a less festooned elastica lamina in adult mice   (MGI Ref ID J:94047)
    • disorganized tunica media in adult mice   (MGI Ref ID J:94047)
    • discontinuous layers of noncohesive smooth muscle cells in the caudal artery of adult mice   (MGI Ref ID J:94047)
    • normal endothelial cells   (MGI Ref ID J:94047)
    • arterial defects are observed in all the organs   (MGI Ref ID J:94047)
    • major elastic arteries of the trunk are normal   (MGI Ref ID J:94047)
    • poor arterial differentiation
      • thin, irregular, and overlapping cytoplasmic processes   (MGI Ref ID J:94047)
      • form abnormal clusters of poorly oriented cells   (MGI Ref ID J:94047)
      • irregular shape of vascular smooth muscle   (MGI Ref ID J:94047)
      • thin cytoplasmic expansions   (MGI Ref ID J:94047)
      • marked reduction of dense plaques and dense bodies   (MGI Ref ID J:94047)
      • no defects in cell proliferation and cell death   (MGI Ref ID J:94047)
      • normal arterial identity of endothelial cells   (MGI Ref ID J:94047)
  • abnormal blood circulation
    • Angiotensin II infusion induces normal blood pressure response   (MGI Ref ID J:94047)
    • Angiotensin II infusion induces strongly defective cerebral blood flow (CBF)   (MGI Ref ID J:94047)
    • normal systolic, diastolic, and mean arterial pressure (MABP)   (MGI Ref ID J:94047)
    • in spite of arterial defects, no tissue damage is seen   (MGI Ref ID J:94047)
    • abnormal vascular resistance
      • Angiotensin II infusion induces strongly defective cerebrovascular resistance (CVR) responses   (MGI Ref ID J:94047)
  • abnormal vascular smooth muscle morphology
    • vascular smooth muscle cells coat is thinner   (MGI Ref ID J:94047)
    • frequently overlapped, thin elongated cytoplasmic processes   (MGI Ref ID J:94047)
  • muscle phenotype
  • abnormal vascular smooth muscle morphology
    • vascular smooth muscle cells coat is thinner   (MGI Ref ID J:94047)
    • frequently overlapped, thin elongated cytoplasmic processes   (MGI Ref ID J:94047)
View Research Applications

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

Cardiovascular Research
Vascular Defects

Developmental Biology Research
Internal/Organ Defects
      vasculature

Research Tools
Developmental Biology Research

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Notch3tm1Grid
Allele Name targeted mutation 1, Tom Gridley
Allele Type Targeted (knock-out)
Common Name(s) Notch3d1;
Mutation Made By Tom Gridley,   Maine Medical Center Research Institute
Strain of Origin129S1/Sv-Oca2<+> Tyr<+> Kitl<+>
Gene Symbol and Name Notch3, notch 3
Chromosome 17
Gene Common Name(s) AW229011; CADASIL; CASIL; IMF2; N3; expressed sequence AW229011; hpbk; humpback;
Molecular Note A 2.5 kb genomic fragment containing sequence encoding EGF repeats 8 through 12 was replaced with a PGK-neo cassette inserted by homologous recombination. The absence of transcript and protein was indicated by Northern and Western blot analysis of homozygous mutant mice. [MGI Ref ID J:87272]

Genotyping

Genotyping Information

Genotyping Protocols

Notch3tm1Grid, Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Krebs LT; Xue Y; Norton CR; Sundberg JP; Beatus P; Lendahl U; Joutel A; Gridley T. 2003. Characterization of Notch3-deficient mice: normal embryonic development and absence of genetic interactions with a Notch1 mutation. Genesis 37(3):139-43. [PubMed: 14595837]  [MGI Ref ID J:87272]

Additional References

Notch3tm1Grid related

Anthony TE; Mason HA; Gridley T; Fishell G; Heintz N. 2005. Brain lipid-binding protein is a direct target of Notch signaling in radial glial cells. Genes Dev 19(9):1028-33. [PubMed: 15879553]  [MGI Ref ID J:98262]

Belin de Chantemele EJ; Retailleau K; Pinaud F; Vessieres E; Bocquet A; Guihot AL; Lemaire B; Domenga V; Baufreton C; Loufrani L; Joutel A; Henrion D. 2008. Notch3 is a major regulator of vascular tone in cerebral and tail resistance arteries. Arterioscler Thromb Vasc Biol 28(12):2216-24. [PubMed: 18818417]  [MGI Ref ID J:159790]

Domenga V; Fardoux P; Lacombe P; Monet M; Maciazek J; Krebs LT; Klonjkowski B; Berrou E; Mericskay M; Li Z; Tournier-Lasserve E; Gridley T; Joutel A. 2004. Notch3 is required for arterial identity and maturation of vascular smooth muscle cells. Genes Dev 18(22):2730-5. [PubMed: 15545631]  [MGI Ref ID J:94047]

Helbig C; Gentek R; Backer RA; de Souza Y; Derks IA; Eldering E; Wagner K; Jankovic D; Gridley T; Moerland PD; Flavell RA; Amsen D. 2012. Notch controls the magnitude of T helper cell responses by promoting cellular longevity. Proc Natl Acad Sci U S A 109(23):9041-6. [PubMed: 22615412]  [MGI Ref ID J:184840]

Jeannet R; Mastio J; Macias-Garcia A; Oravecz A; Ashworth T; Geimer Le Lay AS; Jost B; Le Gras S; Ghysdael J; Gridley T; Honjo T; Radtke F; Aster JC; Chan S; Kastner P. 2010. Oncogenic activation of the Notch1 gene by deletion of its promoter in Ikaros-deficient T-ALL. Blood 116(25):5443-54. [PubMed: 20829372]  [MGI Ref ID J:167410]

Li X; Zhang X; Leathers R; Makino A; Huang C; Parsa P; Macias J; Yuan JX; Jamieson SW; Thistlethwaite PA. 2009. Notch3 signaling promotes the development of pulmonary arterial hypertension. Nat Med 15(11):1289-97. [PubMed: 19855400]  [MGI Ref ID J:154293]

Li Y; Takeshita K; Liu PY; Satoh M; Oyama N; Mukai Y; Chin MT; Krebs L; Kotlikoff MI; Radtke F; Gridley T; Liao JK. 2009. Smooth muscle Notch1 mediates neointimal formation after vascular injury. Circulation 119(20):2686-92. [PubMed: 19433762]  [MGI Ref ID J:166426]

Liu H; Zhang W; Kennard S; Caldwell RB; Lilly B. 2010. Notch3 is critical for proper angiogenesis and mural cell investment. Circ Res 107(7):860-70. [PubMed: 20689064]  [MGI Ref ID J:178201]

Mason HA; Rakowiecki SM; Gridley T; Fishell G. 2006. Loss of notch activity in the developing central nervous system leads to increased cell death. Dev Neurosci 28(1-2):49-57. [PubMed: 16508303]  [MGI Ref ID J:112183]

Mason HA; Rakowiecki SM; Raftopoulou M; Nery S; Huang Y; Gridley T; Fishell G. 2005. Notch signaling coordinates the patterning of striatal compartments. Development 132(19):4247-58. [PubMed: 16120638]  [MGI Ref ID J:101735]

Monet M; Domenga V; Lemaire B; Souilhol C; Langa F; Babinet C; Gridley T; Tournier-Lasserve E; Cohen-Tannoudji M; Joutel A. 2007. The archetypal R90C CADASIL-NOTCH3 mutation retains NOTCH3 function in vivo. Hum Mol Genet 16(8):982-92. [PubMed: 17331978]  [MGI Ref ID J:121707]

Morimoto M; Nishinakamura R; Saga Y; Kopan R. 2012. Different assemblies of Notch receptors coordinate the distribution of the major bronchial Clara, ciliated and neuroendocrine cells. Development 139(23):4365-73. [PubMed: 23132245]  [MGI Ref ID J:190886]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX10

Colony Maintenance

Breeding & HusbandryWhen maintaining a live colony, these mice can be bred as homozygotes.
Mating SystemHomozygote x Homozygote         (Female x Male)   11-DEC-12
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $195.00Female or MaleHomozygous for Notch3tm1Grid  
Price per Pair (US dollars $)Pair Genotype
$390.00Homozygous for Notch3tm1Grid x Homozygous for Notch3tm1Grid  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $253.50Female or MaleHomozygous for Notch3tm1Grid  
Price per Pair (US dollars $)Pair Genotype
$507.00Homozygous for Notch3tm1Grid x Homozygous for Notch3tm1Grid  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1500 unique mouse models across a vast array of research areas. Breeding colonies provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. If a Repository strain is not immediately available, then within 2 to 3 business days, you will receive an estimated availability timeframe for your inquiry or order along with various delivery options. Repository strains typically are delivered at 4 to 8 weeks of age and will not exceed 12 weeks of age on the day of shipping. We will note and try to accommodate requests for specific ages of Repository strains but cannot guarantee provision of these strains at specific ages. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, please let us know.

Control Information

  Control
   101043 B6129SF1/J (approximate)
   101045 B6129SF2/J (approximate)
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

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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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