Stock B6;129-Ptprc<tm1Holm>/H

FESA Number

1622

Strain Name

B6;129-Ptprc<tm1Holm>/H

Mutation Strain Type

Mutation Type Mutation Subtype Strain Type
IMSR - Targeted mutation IMSR - unclassified

Attribution

Institution name Depositor / Originator Name
Babraham Institute Originator Retained
Babraham Institute Depositor Retained

Gene/Allele Information

Allele Name Allele MGI ID Gene Name Gene MGI ID Chromosome
Ptprc<tm1Holm> MGI:1857277 Ptprc MGI:97810 1

Phenotypic Description

Defective lymphocyte development, more severe in T lineage, severe combined immunodeficiency.

Orphanet Disorders

OrphaNet ID Name Url
169 157 T-B+ severe combined immunodeficiency due to CD45 deficiency http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=169157

Publications

Displaying 1 - 10 of 27 Stock Publications
Pubmed ID Authors Title Journal
10970857 Baker M Development of T-leukaemias in CD45 tyrosine phosphatase-deficient mutant lck mice. EMBO J (2 000) 19:4644-54
16771860 Piercy J CD45 negatively regulates tumour necrosis factor and interleukin-6 production in dendritic cells. Immunology (2 006) 118:250-6
8666928 Byth KF CD45-null transgenic mice reveal a positive regulatory role for CD45 in early thymocyte development, in the selection of CD4+CD8+ thymocytes, and B cell maturation. J Exp Med (1 996) 183:1707-18
10458749 Pingel S The CD45 tyrosine phosphatase regulates CD3-induced signal transduction and T cell development in recombinase-deficient mice: restoration of pre-TCR function by active p56(lck). Eur J Immunol (1 999) 29:2376-84
24068938 Caignard G Genome-wide mouse mutagenesis reveals CD45-mediated T cell function as critical in protective immunity to HSV-1. PLoS Pathog (2 013) 9:e1003637
18448457 Salmond RJ CD4+ T cell hyper-responsiveness in CD45 transgenic mice is independent of isoform. Int Immunol (2 008) 20:819-27
12612075 Davidson D Phosphorylation-dependent regulation of T-cell activation by PAG/Cbp, a lipid raft-associated transmembrane adaptor. Mol Cell Biol (2 003) 23:2017-28
9190928 Stone JD Aberrant TCR-mediated signaling in CD45-null thymocytes involves dysfunctional regulation of Lck, Fyn, TCR-zeta, and ZAP-70. J Immunol (1 997) 158:5773-82
16517710 Dawes R Combinations of CD45 isoforms are crucial for immune function and disease. J Immunol (2 006) 176:3417-25
23936270 St-Pierre J A role for the protein tyrosine phosphatase CD45 in macrophage adhesion through the regulation of paxillin degradation. PLoS One (2 013) 8:e71531

Pages

Orphanet Categories

Rare immune disease

EMMA ID

EM:01736

Genetic Status

GMO

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