Mouse Genome Informatics
hm
    Coro1aptcd/Coro1aptcd
B6.CTS-Coro1aptcd
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
immune system
• the percentage of mature (CD69loCD62Lhi) single-positive thymocytes is about doubled in these mice
• similar accumulations of mature T cells in the thymus occurs in irradiated wild-type mice reconstituted with mutant bone marrow
• migrating T cells have irregularly shaped protrusions that are often larger in size than controls
• CD4+ T cell numbers in the periphery are decreased around 10-fold
• even more dramatic reductions are observed in irradiated wild-type mice reconstituted with mutant bone marrow
• CD8+ T cell numbers in the periphery are decreased around 5-fold
• even more dramatic reductions are observed in irradiated wild-type mice reconstituted with mutant bone marrow
• in vitro transwell assays demonstrate migration defects in thymic CD4+ T cells' response to sphingosine 1-phosphate, CCL21, and CXCL12
• similar defects are observed in the migratory response of CD4+CD8+ T cells to CXCL12 and of splenic CD4+ T cells to CCL21
• mutant T cells co-transferred with wild-type T cells into wild-type hosts are found in lower proportions in lymph nodes indicating a defect in lymph node entry
• mutant T cells have a 3-fold less ability to egress out of a wild-type lymph node over a 20 hour period
• T cells within a lymph node move at a lower median velocity of 6.6 microm/min compared to 9.5 micormeter/minute for wild-type
• T cells within a lymph node also have larger turning angles and less-directed paths than their wild-type counterparts
• na´ve CD4+ T cells have about 3-fold higher rates of apoptosis in the spleen
• upon CD3 activation, T cells flux less Ca2+ than wild-type controls
• however, similar Ca2+ flux results as controls are observed when CD4 stimulation is included

hematopoietic system
• the percentage of mature (CD69loCD62Lhi) single-positive thymocytes is about doubled in these mice
• similar accumulations of mature T cells in the thymus occurs in irradiated wild-type mice reconstituted with mutant bone marrow
• migrating T cells have irregularly shaped protrusions that are often larger in size than controls
• CD4+ T cell numbers in the periphery are decreased around 10-fold
• even more dramatic reductions are observed in irradiated wild-type mice reconstituted with mutant bone marrow
• CD8+ T cell numbers in the periphery are decreased around 5-fold
• even more dramatic reductions are observed in irradiated wild-type mice reconstituted with mutant bone marrow
• in vitro transwell assays demonstrate migration defects in thymic CD4+ T cells' response to sphingosine 1-phosphate, CCL21, and CXCL12
• similar defects are observed in the migratory response of CD4+CD8+ T cells to CXCL12 and of splenic CD4+ T cells to CCL21
• mutant T cells co-transferred with wild-type T cells into wild-type hosts are found in lower proportions in lymph nodes indicating a defect in lymph node entry
• mutant T cells have a 3-fold less ability to egress out of a wild-type lymph node over a 20 hour period
• T cells within a lymph node move at a lower median velocity of 6.6 microm/min compared to 9.5 micormeter/minute for wild-type
• T cells within a lymph node also have larger turning angles and less-directed paths than their wild-type counterparts
• na´ve CD4+ T cells have about 3-fold higher rates of apoptosis in the spleen
• upon CD3 activation, T cells flux less Ca2+ than wild-type controls
• however, similar Ca2+ flux results as controls are observed when CD4 stimulation is included

cellular
• na´ve CD4+ T cells have about 3-fold higher rates of apoptosis in the spleen

Mouse Models of Human Disease
OMIM IDRef(s)
Severe Combined Immunodeficiency, Autosomal Recessive, T Cell-Negative, B Cell-Positive, Nk Cell-Positive 608971 J:141431