Strain Name:

B6.129S1-Jag1tm1Grid/J

Stock Number:

010616

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Availability:

Cryopreserved - Ready for recovery

These Jag1dDSL, or Jag null, mutant mice may be useful in studies of Alagille Syndrome 1.

Description

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 Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
GenerationN10pN1
Generation Definitions
 
Donating Investigator Tom Gridley,   Maine Medical Center Research Institute

Description
Mice that are heterozygous for the targeted mutation are viable, fertile, and normal in size. No gene product (mRNA or protein) is detected by RT-PCR or Western blot analysis of homozygous embryos, aged embryonic day 10. Homozygotes have an embryonic lethal phenotype, with defective vasculature formation in the embryo and yolk sac and widespread hemorrhaging at embryonic day 10.5. Heterozygotes exhibit iris coloboma, irregular/off center pupils and corneal opacity.

Development
A targeting vector containing a neo cassette was used to delete 5kb of sequence at the 5' end of the gene, which encodes the C-terminal part of the DSL domain. 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 mice were backcrossed to C57BL/6J for 10 generations.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  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
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
010547   B6;129S1-Notch3tm1Grid/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)1HCeb/OveJ
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 other alleles of Jag1
010618   B6;129S-Jag1tm2Grid/J
View Strains carrying other alleles of Jag1     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- No similarity to the expected human disease phenotype was found. One or more human genes are associated with this human disease. The mouse genotype may involve mutations to orthologs of one or more of these genes, but the phenotype did not resemble the disease.
Alagille Syndrome 1; ALGS1
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Jagged 1; JAG1   (JAG1)
Tetralogy of Fallot; TOF   (JAG1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

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

Jag1tm1Grid/Jag1+

        involves: 129S1/Sv * C57BL/6 * FVB
  • vision/eye phenotype
  • abnormal eye morphology
    • eye phenotypes are described as fully penetrant on an 80% C57BL/6 genetic background and incompletely penetrant on a mixed background   (MGI Ref ID J:54907)
    • abnormal placement of pupils   (MGI Ref ID J:54907)
    • corneal opacity   (MGI Ref ID J:54907)
    • iris coloboma
      • iris coloboma   (MGI Ref ID J:54907)
    • irregularly shaped pupil   (MGI Ref ID J:54907)

Jag1tm1Grid/Jag1tm1Grid

        involves: 129S1/Sv * C57BL/6 * FVB
  • mortality/aging
  • complete embryonic lethality during organogenesis
    • death occurs at approximately E10.5 due to widespread hemorrhages   (MGI Ref ID J:54907)
    • normal appearance at E9.5, and widespread necrosis is observed at E11.5   (MGI Ref ID J:54907)
    • no defects in somitogenesis are observed   (MGI Ref ID J:54907)
  • embryogenesis phenotype
  • abnormal vitelline vascular remodeling
    • large blood vessels are absent from the yolk sacs   (MGI Ref ID J:54907)
  • pale yolk sac
    • a pale yolk sac is seen at E10.5   (MGI Ref ID J:54907)
  • cardiovascular system phenotype
  • abnormal vascular development
    • the vascular network in the forebrain of mutant embryos is not as well developed as in controls   (MGI Ref ID J:54907)
    • large blood vessels of the head have an abnormal appearance and a reduced diameter   (MGI Ref ID J:54907)
    • the primary vascular plexus of the yolk sac does not remodel into large blood vessels   (MGI Ref ID J:54907)
    • abnormal vitelline vascular remodeling
      • large blood vessels are absent from the yolk sacs   (MGI Ref ID J:54907)
  • hemorrhage
    • widespread, including embryonic and extraembryonic tissues observed at E10.5   (MGI Ref ID J:54907)
View Research Applications

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

Cardiovascular Research
Vascular Defects

Developmental Biology Research
Embryonic Lethality (Homozygous)
Eye Defects
Internal/Organ Defects
      vasculature

Sensorineural Research
Eye Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Jag1tm1Grid
Allele Name targeted mutation 1, Tom Gridley
Allele Type Targeted (Null/Knockout)
Common Name(s) Jag1dDSL; Jag1del1; Jag1null;
Mutation Made By Tom Gridley,   Maine Medical Center Research Institute
Strain of Origin129S1/Sv-Oca2<+> Tyr<+> Kitl<+>
Gene Symbol and Name Jag1, jagged 1
Chromosome 2
Gene Common Name(s) ABE2; AGS; AHD; AWS; CD339; Gsfabe2; HJ1; Headturner; Htu; JAGL1; Ozz; Serrate-1; gsf abnormal behavior 2; headturner; ozzy;
Molecular Note A 5kb genomic fragment at the 5' end of the gene was replaced by a neomycin resistance cassette. These deleted sequences include coding regions for the C-terminal portion of the DSL domain. RT-PCR analysis demonstrated that no transcript was detectablein RNA isolated from E10 homozygous embryos, and Western blot analysis on extracts of E10.5 homozygous embryos confirmed that the protein was not expressed. [MGI Ref ID J:54907]

Genotyping

Genotyping Information

Genotyping Protocols

Jag1tm1Grid, Melt Curve Analysis
Jag1tm1Grid, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Additional References

Jag1tm1Grid related

Benedito R; Roca C; Sorensen I; Adams S; Gossler A; Fruttiger M; Adams RH. 2009. The notch ligands Dll4 and Jagged1 have opposing effects on angiogenesis. Cell 137(6):1124-35. [PubMed: 19524514]  [MGI Ref ID J:157345]

Cao Z; Ding BS; Guo P; Lee SB; Butler JM; Casey SC; Simons M; Tam W; Felsher DW; Shido K; Rafii A; Scandura JM; Rafii S. 2014. Angiocrine factors deployed by tumor vascular niche induce B cell lymphoma invasiveness and chemoresistance. Cancer Cell 25(3):350-65. [PubMed: 24651014]  [MGI Ref ID J:210815]

Feng X; Krebs LT; Gridley T. 2010. Patent ductus arteriosus in mice with smooth muscle-specific Jag1 deletion. Development 137(24):4191-9. [PubMed: 21068062]  [MGI Ref ID J:166769]

Fernandez-Valdivia R; Takeuchi H; Samarghandi A; Lopez M; Leonardi J; Haltiwanger RS; Jafar-Nejad H. 2011. Regulation of mammalian Notch signaling and embryonic development by the protein O-glucosyltransferase Rumi. Development 138(10):1925-34. [PubMed: 21490058]  [MGI Ref ID J:171432]

Guiu J; Shimizu R; D'Altri T; Fraser ST; Hatakeyama J; Bresnick EH; Kageyama R; Dzierzak E; Yamamoto M; Espinosa L; Bigas A. 2013. Hes repressors are essential regulators of hematopoietic stem cell development downstream of Notch signaling. J Exp Med 210(1):71-84. [PubMed: 23267012]  [MGI Ref ID J:194596]

Kiernan AE; Ahituv N; Fuchs H; Balling R; Avraham KB; Steel KP; Hrabe de Angelis M. 2001. The Notch ligand Jagged1 is required for inner ear sensory development. Proc Natl Acad Sci U S A 98(7):3873-8. [PubMed: 11259677]  [MGI Ref ID J:72108]

Kiernan AE; Li R; Hawes NL; Churchill GA; Gridley T. 2007. Genetic background modifies inner ear and eye phenotypes of jag1 heterozygous mice. Genetics 177(1):307-11. [PubMed: 17890364]  [MGI Ref ID J:125211]

Kiernan AE; Xu J; Gridley T. 2006. The Notch ligand JAG1 is required for sensory progenitor development in the mammalian inner ear. PLoS Genet 2(1):e4. [PubMed: 16410827]  [MGI Ref ID J:115783]

Loomes KM; Russo P; Ryan M; Nelson A; Underkoffler L; Glover C; Fu H; Gridley T; Kaestner KH; Oakey RJ. 2007. Bile duct proliferation in liver-specific Jag1 conditional knockout mice: effects of gene dosage. Hepatology 45(2):323-30. [PubMed: 17366661]  [MGI Ref ID J:149131]

Lozier J; McCright B; Gridley T. 2008. Notch signaling regulates bile duct morphogenesis in mice. PLoS ONE 3(3):e1851. [PubMed: 18365007]  [MGI Ref ID J:133171]

McCright B; Gao X; Shen L; Lozier J; Lan Y; Maguire M; Herzlinger D; Weinmaster G; Jiang R; Gridley T. 2001. Defects in development of the kidney, heart and eye vasculature in mice homozygous for a hypomorphic Notch2 mutation. Development 128(4):491-502. [PubMed: 11171333]  [MGI Ref ID J:67157]

McCright B; Lozier J; Gridley T. 2002. A mouse model of Alagille syndrome: Notch2 as a genetic modifier of Jag1 haploinsufficiency. Development 129(4):1075-82. [PubMed: 11861489]  [MGI Ref ID J:74574]

Nadeau JH. 2003. Modifier genes and protective alleles in humans and mice. Curr Opin Genet Dev 13(3):290-5. [PubMed: 12787792]  [MGI Ref ID J:88012]

Pan W; Jin Y; Stanger B; Kiernan AE. 2010. Notch signaling is required for the generation of hair cells and supporting cells in the mammalian inner ear. Proc Natl Acad Sci U S A 107(36):15798-803. [PubMed: 20733081]  [MGI Ref ID J:164383]

Robert-Moreno A; Guiu J; Ruiz-Herguido C; Lopez ME; Ingles-Esteve J; Riera L; Tipping A; Enver T; Dzierzak E; Gridley T; Espinosa L; Bigas A. 2008. Impaired embryonic haematopoiesis yet normal arterial development in the absence of the Notch ligand Jagged1. EMBO J 27(13):1886-95. [PubMed: 18528438]  [MGI Ref ID J:137647]

Rodilla V; Villanueva A; Obrador-Hevia A; Robert-Moreno A; Fernandez-Majada V; Grilli A; Lopez-Bigas N; Bellora N; Alba MM; Torres F; Dunach M; Sanjuan X; Gonzalez S; Gridley T; Capella G; Bigas A; Espinosa L. 2009. Jagged1 is the pathological link between Wnt and Notch pathways in colorectal cancer. Proc Natl Acad Sci U S A 106(15):6315-20. [PubMed: 19325125]  [MGI Ref ID J:147760]

Ryan MJ; Bales C; Nelson A; Gonzalez DM; Underkoffler L; Segalov M; Wilson-Rawls J; Cole SE; Moran JL; Russo P; Spinner NB; Kusumi K; Loomes KM. 2008. Bile duct proliferation in Jag1/fringe heterozygous mice identifies candidate modifiers of the Alagille syndrome hepatic phenotype. Hepatology 48(6):1989-97. [PubMed: 19026002]  [MGI Ref ID J:152514]

Underkoffler LA; Carr E; Nelson A; Ryan MJ; Schultz R; Loomes KM. 2013. Microarray data reveal relationship between Jag1 and Ddr1 in mouse liver. PLoS One 8(12):e84383. [PubMed: 24391948]  [MGI Ref ID J:211111]

Xue Y; Gao X; Lindsell CE; Norton CR; Chang B; Hicks C; Gendron-Maguire M ; Rand EB ; Weinmaster G ; Gridley T. 1999. Embryonic lethality and vascular defects in mice lacking the Notch ligand Jagged1. Hum Mol Genet 8(5):723-30. [PubMed: 10196361]  [MGI Ref ID J:54907]

Yao Y; Yao J; Radparvar M; Blazquez-Medela AM; Guihard PJ; Jumabay M; Bostrom KI. 2013. Reducing Jagged 1 and 2 levels prevents cerebral arteriovenous malformations in matrix Gla protein deficiency. Proc Natl Acad Sci U S A 110(47):19071-6. [PubMed: 24191040]  [MGI Ref ID J:202978]

Yen HY; Ting MC; Maxson RE. 2010. Jagged1 functions downstream of Twist1 in the specification of the coronal suture and the formation of a boundary between osteogenic and non-osteogenic cells. Dev Biol 347(2):258-70. [PubMed: 20727876]  [MGI Ref ID J:166632]

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 & HusbandryWhen maintaining a live colony, these mice are bred as heterozygotes. Homozygotes are embryonic lethal due to hemorrhaging.

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


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

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2140.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2782.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  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.
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"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

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