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- W2045460124 abstract "Abstract The human genetic disease X-linked lymphoproliferative disease (XLP), which is caused by mutations in SH2D1A/SAP that encode SLAM-associated protein (SAP), is characterized by an inability to control Epstein-Barr virus (EBV) and hypogammaglobulinemia. It is unclear which aspects of XLP disease are specific to herpesvirus infection and which reflect general immunologic functions performed by SAP. We examined SAP– mice during a chronic LCMV infection, specifically to address the following question: Which SAP deficiency immunologic problems are general, and which are EBV specific? Illness, weight loss, and prolonged viral replication were much more severe in SAP– mice. Aggressive immunopathology was observed. This inability to control chronic LCMV was associated with both CD8 T-cell and B-cell response defects. Importantly, we demonstrate that SAP– CD8 T cells are the primary cause of the immunopathology and clinical illness, because depletion of CD8 T cells blocked disease. This is the first direct demonstration of SAP– CD8 T-cell–mediated immunopathology, confirming 30 years of XLP clinical observations and indirect experimentation. In addition, germinal center formation was extremely defective in chronically infected SAP– animals, and hypogammaglobulinemia was observed. These findings in a chronic viral infection mouse model recapitulate key features of human XLP and clarify SAP's critical role regulating both cellular and humoral immunity." @default.
- W2045460124 created "2016-06-24" @default.
- W2045460124 creator A5012655205 @default.
- W2045460124 creator A5016808814 @default.
- W2045460124 creator A5030160770 @default.
- W2045460124 creator A5059929721 @default.
- W2045460124 creator A5079535759 @default.
- W2045460124 date "2006-11-01" @default.
- W2045460124 modified "2023-09-29" @default.
- W2045460124 title "Hypogammaglobulinemia and exacerbated CD8 T-cell–mediated immunopathology in SAP-deficient mice with chronic LCMV infection mimics human XLP disease" @default.
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- W2045460124 doi "https://doi.org/10.1182/blood-2006-04-018929" @default.
- W2045460124 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16788096" @default.
- W2045460124 hasPublicationYear "2006" @default.
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