SAP Gene Defects In Male Patients Diagnosed With Common Variable Immunodeficiency

The molecular basis of Common Variable Immunodeficiency (CVID) is unknown and the diagnosis requires the exclusion of other diseases known to cause primary antibody deficiency syndromes, such as X-linked lymphoproliferative disease (XLP). XLP is a rare primary immunodeficiency disease in which severe immune dysregulatory phenomena occur, typically after exposure to Epstein–Barr virus (EBV). The defective gene in XLP is SAP (SLAM associated protein, where SLAM is signalling lymphocyte activation molecule), a small SH2 domain containing protein involved in signal transduction events downstream of the SLAM family of receptors. (more…)