Human Predicted to work with:
Synthetic peptide within Human XIAP (N terminal) (phospho S87) conjugated to Keyhole Limpet Haemocyanin (KLH). The exact sequence is proprietary. [Peptide sequence around phosphorylation site of serine 87(K-V-S(p)-P-N)]. Database link: P98170
HepG2 cell extract treated with anisomycin.
Shipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Apoptotic suppressor. Has E3 ubiquitin-protein ligase activity. Mediates the proteasomal degradation of target proteins, such as caspase-3, SMAC or AIFM1. Inhibitor of caspase-3, -7 and -9. Mediates activation of MAP3K7/TAK1, leading to the activation of NF-kappa-B.
Ubiquitous, except peripheral blood leukocytes.
Involvement in disease
Defects in XIAP are the cause of lymphoproliferative syndrome X-linked type 2 (XLP2) [MIM:300635]. XLP is a rare immunodeficiency characterized by extreme susceptibility to infection with Epstein-Barr virus (EBV). Symptoms include severe or fatal mononucleosis, acquired hypogammaglobulinemia, pancytopenia and malignant lymphoma.
Belongs to the IAP family. Contains 3 BIR repeats. Contains 1 RING-type zinc finger.
The first BIR domain is involved in interaction with TAB1/MAP3K7IP1 and is important for dimerization. The second BIR domain is sufficient to inhibit caspase-3 and caspase-7, while the third BIR is involved in caspase-9 inhibition. The interactions with SMAC and PRSS25 are mediated by the second and third BIR domains.
Ubiquitinated and degraded by the proteasome in apoptotic cells. Phosphorylation by PKB/AKT protects XIAP against ubiquitination and protects the protein against proteasomal degradation.