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| 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
- Disease & phenotype
- Genes & alleles
- Genotyping
- Health & care
- References
- Pricing & purchasing
- Terms of use
Description
Strain Information
Type Mutant Stock; Mutant Strain; Targeted Mutation; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Mating System Homozygote x Homozygote (Female x Male) 11-DEC-12 Species laboratory mouse Generation F?+F5 (22-MAY-12)
Generation DefinitionsDonating 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
View Facebase: models (58 strains)
005330 C57BL/6J-Notch3hpbk/GrsrJ
Phenotype
Phenotype Information
- 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)
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 may relate to a genetic background differing from 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
- muscle phenotype
- abnormal vascular smooth muscle morphology
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 Origin | 129S1/Sv-Oca2<+> Tyr<+> Kitl<+> | ||
| Gene Symbol and Name | Notch3, notch 3 | ||
| Chromosome | 17 | ||
| Gene Common Name(s) | AW229011; CADASIL; CASIL; 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 relatedAnthony 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 AX11
Colony Maintenance
Breeding & Husbandry When maintaining a live colony, these mice can be bred as homozygotes. Mating System Homozygote 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 |
|
Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $177.00 Female or Male Homozygous for Notch3tm1Grid
Price per Pair (US dollars $) Pair Genotype $354.00 Homozygous for Notch3tm1Grid x Homozygous for Notch3tm1Grid Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order 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.
| Pricing for International shipping destinations |
|
Live Mice
Price per mouse (US dollars $) Gender Genotypes Provided Individual Mouse $230.10 Female or Male Homozygous for Notch3tm1Grid
Price per Pair (US dollars $) Pair Genotype $460.20 Homozygous for Notch3tm1Grid x Homozygous for Notch3tm1Grid Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order 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.
|
|
|
Standard Supply
Repository-Live. Repository-Live represents an exclusive set of over 1500 unique mouse models maintained at The Jackson Laboratory to support a vast array of research areas. The breeding colonies for Repository Strains provide mice for both large and small orders and fluctuate in size depending on current demand for each strain. Repository-live orders are treated as custom orders. Within 2 business days, we respond to each availability inquiry or order 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.
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's Genotype Promise
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.Ordering Information
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