Extreme lymphoproliferative disease and fatal autoimmune thrombocytopenia in FasL and TRAIL double-deficient mice.

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dc.contributor.author Sedger, Lisa en_US
dc.contributor.author Katewa, Arna en_US
dc.contributor.author Pettersen, Ann en_US
dc.contributor.author Osvath, S en_US
dc.contributor.author Farrell, Geoffrey en_US
dc.contributor.author Stewart, Greame en_US
dc.contributor.author Bendall, L en_US
dc.contributor.author Alexander, Stephen en_US
dc.contributor.editor en_US
dc.date.accessioned 2011-02-07T06:24:54Z
dc.date.available 2011-02-07T06:24:54Z
dc.date.issued 2010 en_US
dc.identifier 2009005242 en_US
dc.identifier.citation Sedger Lisa et al. 2010, 'Extreme lymphoproliferative disease and fatal autoimmune thrombocytopenia in FasL and TRAIL double-deficient mice.', American Society of Hematology, vol. 115, no. 16, pp. 3258-3268. en_US
dc.identifier.issn 0006-4971 en_US
dc.identifier.other C1 en_US
dc.identifier.uri http://hdl.handle.net/10453/13809
dc.description.abstract To delineate the relative roles of tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) and Fas ligand in lymphocyte biology and lymphoproliferative disease, we generated mice defective in both molecules. B6.GT mice develop severe polyclonal lymphoproliferative disease because of accumulating CD3+CD4?CD8?B220+ T cells, CD4+ and CD8+ T cells, and follicular B cells, and mice die prematurely from extreme lymphocytosis, thrombocytopenia, and hemorrhage. Accumulating lymphocytes resembled antigen-experienced lymphocytes, consistent with the maximal resistance of B6.GT CD4+ and CD8+ T cell to activation-induced cell death. More specifically, we show that TRAIL contributes to Fas ligand-mediated activation-induced cell death and controls lymphocyte apoptosis in the presence of interferon-{gamma} once antigen stimulation is removed. Furthermore, dysregulated lymphocyte homeostasis results in the production of anti-DNA and rheumatoid factor autoantibodies, as well as antiplatelet IgM and IgG causing thrombocytopenia. Thus, B6.GT mice reveal new roles for TRAIL in lymphocyte homeostasis and autoimmune lymphoproliferative syndromes and are a model of spontaneous idiopathic thrombocytopenia purpura secondary to lymphoproliferative disease en_US
dc.language en_US
dc.publisher American Society of Hematology en_US
dc.relation.isbasedon http://dx.doi.org/10.1182/blood-2009-11-255497 en_US
dc.title Extreme lymphoproliferative disease and fatal autoimmune thrombocytopenia in FasL and TRAIL double-deficient mice. en_US
dc.parent Blood en_US
dc.journal.volume 115 en_US
dc.journal.number 16 en_US
dc.publocation USA en_US
dc.identifier.startpage 3258 en_US
dc.identifier.endpage 3268 en_US
dc.cauo.name SCI.Medical and Molecular Biosciences en_US
dc.conference Verified OK en_US
dc.for 060100 en_US
dc.personcode 102397 en_US
dc.personcode 103839 en_US
dc.personcode 107692 en_US
dc.personcode 0000062221 en_US
dc.personcode 0000049500 en_US
dc.personcode 0000049504 en_US
dc.personcode 0000062222 en_US
dc.personcode 0000049503 en_US
dc.percentage 100 en_US
dc.classification.name Biochemistry and Cell Biology en_US
dc.classification.type FOR-08 en_US
dc.edition en_US
dc.custom en_US
dc.date.activity en_US
dc.location.activity en_US
dc.description.keywords NA en_US
dc.staffid en_US

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