C57BL/6JSfdAnu-Zap70mrtle/ApbH

Status

Available to order

EMMA IDEM:02173
International strain nameC57BL/6JSfdAnu-Zap70mrtle/ApbH
Alternative nameMr T-Less, C57BL/6Apb-Zap70/Apb
Strain typeInduced Mutant Strains : Chemically-induced
Allele/Transgene symbolZap70mrtle,
Gene/Transgene symbolZap70

Information from provider

ProviderChris Goodnow
Provider affiliationThe Australian National University
Genetic informationThis strain carries a T to C at nucleotide 1601 (W504R) and was identified by systematically screening the mouse genome for autoimmune regulators. This mutant strain develops fewer CD4 and CD8 positive cells and autoimmunity. The strain has a similar phenotype to the published gene knockouts mutants. This strain may assist in the understanding of the molecular requirements for the development of autoimmune disease.
Phenotypic informationThis strain was identified by severe reduction in T cell numbers in blood, but B cells are normal. Both CD4+ and CD8+ T cell populations were almost completely absent in peripheral blood. Therefore, no alpha/beta T cells but have an increase in gamma/delta T cells. Normal numbers of double-negative (DN) and double-positive (DP) T cells. This implies that they have a block at DP stage and mice are unable to perform positive thymocyte selection. This is intrinsic to T cells and not the microenvironment. Cells are unable to pass thymic selection steps and unable to up-regulate the T cell receptor (TCR). There is also reduced signalling through TCR. Zap70 protein expression of mrt/mrt thymocytes was 25% of wildtype thymocytes. The kinase domain is also affected by this mutation - kinase assays not performed. Heterozygous mice, mrt/+ have normal levels of CD4+ and CD8+ thymocytes but Zap70 protein expression is 2 fold lower.
ReferencesNone available

Information from EMMA

Archiving centreMary Lyon Centre at MRC Harwell, Oxford, United Kingdom

Disease and phenotype information

Orphanet associated rare diseases, based on orthologous gene matching

    • Combined immunodeficiency due to ZAP70 deficiency / Orphanet_911
MGI phenotypes (allele matching)
  • arrested T cell differentiation / MGI
  • abnormal T cell subpopulation ratio / MGI
  • abnormal positive T cell selection / MGI
  • decreased T cell number / MGI
  • absent T cells / MGI
  • abnormal double-positive T cell morphology / MGI
  • abnormal regulatory T cell number / MGI
  • increased double-positive T cell number / MGI
  • decreased CD4-positive, alpha beta T cell number / MGI
  • decreased CD8-positive, alpha-beta T cell number / MGI
  • abnormal CD4-positive, alpha beta T cell morphology / MGI

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Availabilities

Requesting frozen sperm or embryos is generally advisable wherever possible, in order to minimise the shipment of live mice.

  • Frozen sperm. Delivered in 4 weeks (after paperwork in place). €1740*
  • Rederivation of mice from frozen stock, delivery time available upon request . €3880*

Due to the dynamic nature of our processes strain availability may change at short notice. The local repository manager will advise you in these circumstances.

* In addition users have to cover all the shipping costs (including the cost for returning dry-shippers, where applicable).

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Practical information

Example health report
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