Mice deficient in the X‐linked lymphoproliferative disease gene <i>sap</i> exhibit increased susceptibility to murine gammaherpesvirus‐68 and hypo‐gammaglobulinemia

Abstract excerpt

X-linked lymphoproliferative disease is characterized by immune dysregulation and uncontrolled lymphoproliferation on exposure to Epstein-Barr virus (EBV). This disease has been attributed to mutations in the SAP gene (also denominated as SH2D1A or DSHP). To delineate the role of SAP in the pathophysiology of X-linked lymphoproliferative disease, a strain of sap-deficient mice has been generated by deleting exon...

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