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X-linked inhibitor of apoptosis protein (XIAP) is an apoptotic suppressor. XIAP has E3 ubiquitin-protein ligase activity. XIAP mediates the proteasomal degradation of target proteins, such as caspase-3, SMAC or AIFM1. XIAP is an inhibitor of caspase-3, -7 and -9. XIAP mediates activation of MAP3K7/TAK1, leading to the activation of NF-kappa-B. Defects in XIAP are the cause of lymphoproliferative syndrome X-linked type 2 (XLP2). XLP is a rare immunodeficiency characterized by extreme susceptibility to infection with Epstein-Barr virus (EBV).