![]() ![]() Signaling lymphocytic activation molecule-associated protein (SAP encoded by SH2D1A) is mutated in XLP1, and X-linked inhibitor of apoptosis (XIAP encoded by BIRC4) is mutated in XLP2. X-linked lymphoproliferative disease (XLP1), described in the mid-1970s and molecularly defined in 1998, and XLP2, reported in 2006, are prematurely lethal genetic immunodeficiencies that share susceptibility to overwhelming inflammatory responses to certain infectious triggers.
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