Homo sapiens Protein: XIAP | |||||||||||||||||||||||||||||||||||
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Summary | |||||||||||||||||||||||||||||||||||
InnateDB Protein | IDBP-380315.5 | ||||||||||||||||||||||||||||||||||
Last Modified | 2014-10-13 [Report errors or provide feedback] | ||||||||||||||||||||||||||||||||||
Gene Symbol | XIAP | ||||||||||||||||||||||||||||||||||
Protein Name | X-linked inhibitor of apoptosis | ||||||||||||||||||||||||||||||||||
Synonyms | |||||||||||||||||||||||||||||||||||
Species | Homo sapiens | ||||||||||||||||||||||||||||||||||
Ensembl Protein | ENSP00000395230 | ||||||||||||||||||||||||||||||||||
InnateDB Gene | IDBG-85142 (XIAP) | ||||||||||||||||||||||||||||||||||
Protein Structure | |||||||||||||||||||||||||||||||||||
UniProt Annotation | |||||||||||||||||||||||||||||||||||
Function | Multi-functional protein which regulates not only caspases and apoptosis, but also modulates inflammatory signaling and immunity, copper homeostasis, mitogenic kinase signaling, cell proliferation, as well as cell invasion and metastasis. Acts as a direct caspase inhibitor. Directly bind to the active site pocket of CASP3 and CASP7 and obstructs substrate entry. Inactivates CASP9 by keeping it in a monomeric, inactive state. Acts as an E3 ubiquitin-protein ligase regulating NF-kappa-B signaling and the target proteins for its E3 ubiquitin-protein ligase activity include: RIPK1, CASP3, CASP7, CASP8, CASP9, MAP3K2/MEKK2, DIABLO/SMAC, AIFM1, CCS and BIRC5/survivin. Ubiquitinion of CCS leads to enhancement of its chaperone activity toward its physiologic target, SOD1, rather than proteasomal degradation. Ubiquitinion of MAP3K2/MEKK2 and AIFM1 does not lead to proteasomal degradation. Plays a role in copper homeostasis by ubiquitinationg COMMD1 and promoting its proteasomal degradation. Can also function as E3 ubiquitin-protein ligase of the NEDD8 conjugation pathway, targeting effector caspases for neddylation and inactivation. Regulates the BMP signaling pathway and the SMAD and MAP3K7/TAK1 dependent pathways leading to NF-kappa-B and JNK activation. Acts as an important regulator of innate immune signaling via regulation of Nodlike receptors (NLRs). Protects cells from spontaneous formation of the ripoptosome, a large multi-protein complex that has the capability to kill cancer cells in a caspase-dependent and caspase-independent manner. Suppresses ripoptosome formation by ubiquitinating RIPK1 and CASP8. Acts as a positive regulator of Wnt signaling and ubiquitinates TLE1, TLE2, TLE3, TLE4 and AES. Ubiquitination of TLE3 results in inhibition of its interaction with TCF7L2/TCF4 thereby allowing efficient recruitment and binding of the transcriptional coactivator beta- catenin to TCF7L2/TCF4 that is required to initiate a Wnt-specific transcriptional program. {ECO:0000269PubMed:11447297, ECO:0000269PubMed:12121969, ECO:0000269PubMed:14645242, ECO:0000269PubMed:14685266, ECO:0000269PubMed:17560374, ECO:0000269PubMed:17967870, ECO:0000269PubMed:19473982, ECO:0000269PubMed:20154138, ECO:0000269PubMed:21145488, ECO:0000269PubMed:22103349, ECO:0000269PubMed:22304967, ECO:0000269PubMed:9230442}. | ||||||||||||||||||||||||||||||||||
Subcellular Localization | Cytoplasm. Nucleus. Note=TLE3 promotes its nuclear localization. | ||||||||||||||||||||||||||||||||||
Disease Associations | Lymphoproliferative syndrome, X-linked, 2 (XLP2) [MIM:300635]: 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. {ECO:0000269PubMed:17080092}. Note=The disease is caused by mutations affecting the gene represented in this entry. | ||||||||||||||||||||||||||||||||||
Tissue Specificity | Ubiquitous, except peripheral blood leukocytes. | ||||||||||||||||||||||||||||||||||
Comments | |||||||||||||||||||||||||||||||||||
Interactions | |||||||||||||||||||||||||||||||||||
Number of Interactions |
This gene and/or its encoded proteins are associated with 221 experimentally validated interaction(s) in this database.
They are also associated with 11 interaction(s) predicted by orthology.
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Gene Ontology | |||||||||||||||||||||||||||||||||||
Molecular Function |
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Biological Process |
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Cellular Component |
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Protein Structure and Domains | |||||||||||||||||||||||||||||||||||
PDB ID | |||||||||||||||||||||||||||||||||||
InterPro |
IPR001370
Baculoviral inhibition of apoptosis protein repeat IPR001841 Zinc finger, RING-type |
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PFAM |
PF00653
PF13639 PF14634 |
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PRINTS | |||||||||||||||||||||||||||||||||||
PIRSF | |||||||||||||||||||||||||||||||||||
SMART |
SM00238
SM00184 |
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TIGRFAMs | |||||||||||||||||||||||||||||||||||
Post-translational Modifications | |||||||||||||||||||||||||||||||||||
Modification | |||||||||||||||||||||||||||||||||||
Cross-References | |||||||||||||||||||||||||||||||||||
SwissProt | P98170 | ||||||||||||||||||||||||||||||||||
PhosphoSite | PhosphoSite-P98170 | ||||||||||||||||||||||||||||||||||
TrEMBL | B1AKU2 | ||||||||||||||||||||||||||||||||||
UniProt Splice Variant | |||||||||||||||||||||||||||||||||||
Entrez Gene | 331 | ||||||||||||||||||||||||||||||||||
UniGene | Hs.718045 | ||||||||||||||||||||||||||||||||||
RefSeq | NP_001191330 | ||||||||||||||||||||||||||||||||||
HUGO | HGNC:592 | ||||||||||||||||||||||||||||||||||
OMIM | 300079 | ||||||||||||||||||||||||||||||||||
CCDS | CCDS14606 | ||||||||||||||||||||||||||||||||||
HPRD | 02094 | ||||||||||||||||||||||||||||||||||
IMGT | |||||||||||||||||||||||||||||||||||
EMBL | AL022156 AL121601 AL591583 AY886519 BC032729 CH471107 U32974 U45880 | ||||||||||||||||||||||||||||||||||
GenPept | AAC50373 AAC50518 AAH32729 AAW62257 CAB95312 EAX11858 EAX11859 EAX11860 EAX11861 | ||||||||||||||||||||||||||||||||||