Strain Name:

B6.129-Juptm1Kem/J

Stock Number:

003360

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Availability:

Cryopreserved - Ready for recovery

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.
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Additional information on Congenic nomenclature.
Specieslaboratory mouse
Background Strain C57BL/6
Donor Strain 129X1 x 129S1 via R1 (+Kitl-SlJ) ES cell line
 
Donating InvestigatorProf. Dr. RolfK Kemler,   Max Planck Institute for Immunobiology

Description
Plakoglobin Jup null-mutant embryos die from embryonic day 10.5 onward due to severe heart defects. Some mutant embryos develop further, especially on a C57BL/6 background. These mice die around birth, presumably due to cardiac dysfunction, and with skin blistering and subcorneal acantholysis. The skin phenotype in plakoglobin-deficient mice is reminiscent of the human blistering disease, epidermolytic hyperkeratosis.

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of Jup
003334   B6.129-Juptm1Ruiz/J
022821   B6N(Cg)-Juptm1.1(KOMP)Vlcg/J
017575   STOCK Juptm1.1Glr/J
View Strains carrying other alleles of Jup     (3 strains)

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.
Arrhythmogenic Right Ventricular Dysplasia, Familial, 12; ARVD12   (JUP)
Naxos Disease   (JUP)
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.

Juptm1Kem/Juptm1Kem

        involves: 129S1/Sv * 129X1/SvJ
  • mortality/aging
  • partial embryonic lethality during organogenesis
    • about 50% die between E9.5 and E12.5, with the proportion of dead mutants increasing from E12.5 onward, especially on the 129/Sv background, while some survive longer (to E17.5) on the C57BL/6 background   (MGI Ref ID J:38047)
  • embryogenesis phenotype
  • small placenta
    • small placenta, with little blood supply   (MGI Ref ID J:38047)
  • cardiovascular system phenotype
  • abnormal blood circulation
    • coagulated blood is found in ventricles, atria, and pericardium   (MGI Ref ID J:38047)
    • hemopericardium
      • coagulated blood is found in the pericardium   (MGI Ref ID J:38047)
  • abnormal heart atrium morphology
    • poorly developed atrial trabeculae   (MGI Ref ID J:38047)
  • abnormal heart development
    • heart is structurally less well developed by E10.5 and E112.5   (MGI Ref ID J:38047)
    • abnormal atrioventricular cushion morphology
      • poorly developed endocardial cushion   (MGI Ref ID J:38047)
  • absent cardiac desmosomes
    • at E12.5, hearts show reduced number of desmosomes, less dense plaques, and atypical appearance of desmogleas   (MGI Ref ID J:38047)
  • thin myocardium
    • heart wall is thin and weak   (MGI Ref ID J:38047)
  • trabecula carnea hypoplasia   (MGI Ref ID J:38047)
  • liver/biliary system phenotype
  • small liver
    • small liver with a thin liver capsule and little blood supply   (MGI Ref ID J:38047)
  • muscle phenotype
  • absent cardiac desmosomes
    • at E12.5, hearts show reduced number of desmosomes, less dense plaques, and atypical appearance of desmogleas   (MGI Ref ID J:38047)
  • trabecula carnea hypoplasia   (MGI Ref ID J:38047)
  • homeostasis/metabolism phenotype
  • hemopericardium
    • coagulated blood is found in the pericardium   (MGI Ref ID J:38047)
View Research Applications

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

Dermatology Research
Skin and Hair Texture Defects

Juptm1Kem related

Cardiovascular Research
Heart Abnormalities

Cell Biology Research
Defects in Cell Adhesion Molecules

Developmental Biology Research
Defects in Cell Adhesion Molecules
Embryonic Lethality (Homozygous)
Internal/Organ Defects
      heart

Internal/Organ Research
Heart Abnormalities

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Juptm1Kem
Allele Name targeted mutation 1, Rolf Kemler
Allele Type Targeted (Null/Knockout)
Common Name(s) Pg-; plako-;
Mutation Made ByProf. Dr. RolfK Kemler,   Max Planck Institute for Immunobiology
Strain of Origin(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
ES Cell Line NameR1
ES Cell Line Strain(129X1/SvJ x 129S1/Sv)F1-Kitl<+>
Gene Symbol and Name Jup, junction plakoglobin
Chromosome 11
Gene Common Name(s) AL022715; ARVD12; AW545708; CTNNG; D930025P04Rik; DP3; DPIII; PDGB; PG; PKGB; RIKEN cDNA D930025P04 gene; expressed sequence AL022715; expressed sequence AW545708; gamma-catenin; plakoglobin;
Molecular Note A neomycin resistance cassette replaced exons 3 and 4 of the gene. [MGI Ref ID J:38047]

Genotyping

Genotyping Information

Genotyping Protocols

Juptm1Kem, Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Bierkamp C; Mclaughlin KJ; Schwarz H; Huber O; Kemler R. 1996. Embryonic heart and skin defects in mice lacking plakoglobin. Dev Biol 180(2):780-5. [PubMed: 8954745]  [MGI Ref ID J:38047]

Additional References

Juptm1Kem related

Acehan D; Petzold C; Gumper I; Sabatini DD; Muller EJ; Cowin P; Stokes DL. 2008. Plakoglobin is required for effective intermediate filament anchorage to desmosomes. J Invest Dermatol 128(11):2665-75. [PubMed: 18496566]  [MGI Ref ID J:148138]

Bierkamp C; Schwarz H; Huber O; Kemler R. 1999. Desmosomal localization of beta-catenin in the skin of plakoglobin null-mutant mice. Development 126(2):371-81. [PubMed: 9847250]  [MGI Ref ID J:51129]

Koch U; Wilson A; Cobas M; Kemler R; Macdonald HR; Radtke F. 2008. Simultaneous loss of beta- and gamma-catenin does not perturb hematopoiesis or lymphopoiesis. Blood 111(1):160-4. [PubMed: 17855627]  [MGI Ref ID J:129952]

Todorovic V; Desai BV; Patterson MJ; Amargo EV; Dubash AD; Yin T; Jones JC; Green KJ. 2010. Plakoglobin regulates cell motility through Rho- and fibronectin-dependent Src signaling. J Cell Sci 123(Pt 20):3576-86. [PubMed: 20876660]  [MGI Ref ID J:182813]

Yin T; Getsios S; Caldelari R; Kowalczyk AP; Muller EJ; Jones JC; Green KJ. 2005. Plakoglobin suppresses keratinocyte motility through both cell-cell adhesion-dependent and -independent mechanisms. Proc Natl Acad Sci U S A 102(15):5420-5. [PubMed: 15805189]  [MGI Ref ID J:97837]

Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

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

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* $3300.00
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* $4290.00
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
 
  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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