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Category Symbol Source: HGNC EntrezGene Ensembl GeneCards RNA genes CroW21

GIFtS
-

XIAP Gene

protein-coding GIFtS: 68
GCID: GC0XP122993

X-linked inhibitor of apoptosis

(Previous name: baculoviral IAP repeat-containing 4 )
(Previous symbols: API3, BIRC4)

Explore 116 diseases affiliated with
XIAP via

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(According to ¹HGNC, ²Entrez Gene,
³UniProtKB/Swiss-Prot, ⁴UniProtKB/TrEMBL, ⁵OMIM, ⁶GeneLoc, ⁷Ensembl, ⁸DME, ⁹miRBase, and/or ¹⁰fRNAdb)
About This Section

This gene clusters with an RNA gene
Subcategory (RNA class): lncRNA

Quality score for the ORGUL clustered with this gene is 3

Aliases
X-Linked Inhibitor Of Apoptosis¹ ²		X-Linked IAP² ³
API3¹ ² ³ ⁵		XLP2² ⁵
BIRC4¹ ² ³ ⁵		Baculoviral IAP Repeat-Containing 4¹
HILP¹		ILP1²
Baculoviral IAP Repeat-Containing Protein 4² ³		MIHA²
Inhibitor Of Apoptosis Protein 3² ³		E3 Ubiquitin-Protein Ligase XIAP²
IAP-3² ³		EC 6.3.2.-³
HIAP-3¹		IAP3³
HIAP3¹		ILP³
IAP-Like Protein² ³		X-Linked Inhibitor Of Apoptosis Protein³

External Ids:	HGNC: 592¹	Entrez Gene: 331²	Ensembl: ENSG00000101966⁷	OMIM: 300079⁵	UniProtKB: P98170³

ORGUL members:
	NONCODE:n410669

Export aliases for XIAP gene to outside databases

Previous GC identifers: GC0XP122823 GC0XP112378

(According to Entrez Gene, Tocris Bioscience, Wikipedia's Gene Wiki, PharmGKB,
UniProtKB/Swiss-Prot, and/or UniProtKB/TrEMBL)
About This Section

Entrez Gene summary for XIAP:

This gene encodes a protein that belongs to a family of apoptotic suppressor proteins. Members of this family share a

conserved motif termed, baculovirus IAP repeat, which is necessary for their anti-apoptotic function. This protein

functions through binding to tumor necrosis factor receptor-associated factors TRAF1 and TRAF2 and inhibits apoptosis

induced by menadione, a potent inducer of free radicals, and interleukin 1-beta converting enzyme. This protein also

inhibits at least two members of the caspase family of cell-death proteases, caspase-3 and caspase-7. Mutations in

this gene are the cause of X-linked lymphoproliferative syndrome. Alternate splicing results in multiple transcript

variants. Pseudogenes of this gene are found on chromosomes 2 and 11.(provided by RefSeq, Feb 2011)

UniProtKB/Swiss-Prot: XIAP_HUMAN, P98170

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

summary for XIAP:

Inhibitor of apoptosis (IAPs) are a family of proteins that are the major regulators of apoptosis through

their interaction with the various components of the apoptotic signalling cascades. Originally discovered in

baculovirus, the human homologues are sometimes described as baculovirus IAP repeat containing (BIRC)

proteins. There are a number of different IAPs including neuronal apoptosis inhibitory protein (NAIP or

BIRC1), cellular IAPs (c-IAP-1 and c-IAP-2, or BIRC2 and BIRC3 respectively), X-chromosome linked IAP (XIAP

or BIRC4) and survivin (BIRC5). cIAP-1 and 2 function through interaction with TNF receptors and by binding

to the mitochondrial protein, SMAC, preventing it binding to XIAP. Thought to be the most potent apoptosis

suppressor, XIAP, directly binds and inhibits caspases -3, -7 and -9. Survivin, which also binds to several

caspases, is up-regulated in a many tumour cell types.

Gene Wiki entry for XIAP

(According to GeneLoc and/or HGNC, and/or
Entrez Gene (NCBI build 37),
and/or miRBase,
Genomic Views according to UCSC (hg19) and Ensembl (release 69), Regulatory elements and Epigenetics data according to QIAGEN, SABiosciences, and/or SwitchGear Genomics)
About This Section

RefSeq DNA sequence:

NC_000023.10 NC_018934.1 NT_011786.16

Regulatory elements:

SABiosciences Regulatory transcription factor binding sites in the XIAP gene promoter:
c-Fos Sp1 AP-1 ATF-2 STAT5A c-Jun
Other transcription factors

SwitchGear Promoter luciferase reporter plasmid: XIAP promoter sequence

Search SABiosciences Chromatin IP Primers for XIAP

Epigenetics:

QIAGEN PyroMark CpG Assay predesigned Pyrosequencing DNA Methylation assays in human, mouse, rat XIAP

Genomic Location:
Genomic View: UCSC Golden Path with GeneCards custom track

Entrez Gene cytogenetic band: Xq25 Ensembl cytogenetic band: Xq25 HGNC cytogenetic band: Xq25

XIAP Gene in genomic location: bands according to Ensembl, locations according to (and/or Entrez Gene and/or Ensembl if different)
XIAP gene location

GeneLoc information about chromosome X GeneLoc Exon Structure

GeneLoc location for GC0XP122993: view genomic region (about GC identifiers)

Start:	122,993,574 bp from pter	End:	123,047,829 bp from pter
Size:	54,256 bases	Orientation:	plus strand

(According to ¹UniProtKB, HORDE, neXtProt, Ensembl, and/or Reactome, Modification sites according to ²PhosphoSitePlus, Specific Peptides from DME, Protein expression images according to data from SPIRE MOPED and PaxDb, RefSeq according to NCBI, PDB rendering according to OCA and/or Proteopedia, Recombinant Proteins from EMD Millipore, R&D Systems, GenScript, Enzo Life Sciences, OriGene, Novus Biologicals, Sino Biological, ProSpec, and/or Uscn,
Biochemical Assays by EMD Millipore, R&D Systems, OriGene, GenScript, Cell Signaling Technology, Enzo Life Sciences, and/or Uscn, Ontologies according to Gene Ontology Consortium 01 Mar 2013 and Entrez Gene, Antibodies by EMD Millipore, R&D Systems, GenScript, Cell Signaling Technology, OriGene, Novus Biologicals, Thermo Fisher Scientific, Abcam, and/or Uscn)
About This Section

UniProtKB/Swiss-Prot: XIAP_HUMAN, P98170 (See protein sequence)

Recommended Name: E3 ubiquitin-protein ligase XIAP

Size: 497 amino acids; 56685 Da

Subunit: Monomer, and homodimer. Interacts with DIABLO/SMAC and with PRSS25; these interactions inhibit apoptotic

suppressor activity. Interacts with TAB1/MAP3K7IP1 and AIFM1. Interaction with SMAC hinders binding of TAB1/MAP3K7IP1

and AIFM1. Interacts with TCF25 and COMMD1. Interacts with SEPT4 isoform 6, but not with other SEPT4 isoforms.

Interacts with RIP1, RIP2, RIP3, RIP4, CCS and USP19. Interacts (via BIR 2 domain and BIR 3 domain) with HAX1 (via

C-terminus) and this interaction blocks ubiquitination of XIAP/BIRC4. Interacts with the monomeric form of

BIRC5/survivin. Interacts with TLE3 and TCF7L2/TCF4

Subcellular location: Cytoplasm. Nucleus. Note=TLE3 promotes its nuclear localization

6/32 PDB 3D structures from and Proteopedia for XIAP (see all 32):

1C9Q (3D)

1F9X (3D)

1G3F (3D)

1G73 (3D)

1I3O (3D)

1I4O (3D)

Secondary accessions: D3DTF2 Q9NQ14

Explore the universe of human proteins at neXtProt for XIAP: NX_P98170

Post-translational modifications:

S-Nitrosylation down-regulates its E3 ubiquitin-protein ligase activity¹

Autoubiquitinated and degraded by the proteasome in apoptotic cells¹

Phosphorylation by PKB/AKT protects XIAP against ubiquitination and protects the protein against proteasomal

degradation¹

View modification sites using PhosphoSitePlus²

View neXtProt modification sites for NX_P98170

XIAP Protein expression data from MOPED and PaxDb: About this image

REFSEQ proteins (2 alternative transcripts):

NP_001158.2 NP_001191330.1

ENSEMBL proteins:

ENSP00000405529 ENSP00000400637 ENSP00000360242 ENSP00000347858 ENSP00000395230

Reactome Protein details: P98170
Human Recombinant Protein Products:

	Browse Purified and Recombinant Proteins at EMD Millipore
	R&D Systems Recombinant & Natural Proteins for XIAP
	Browse recombinant and purified proteins available from Enzo Life Sciences
	OriGene Purified Protein: XIAP OriGene Protein Over-expression Lysate: XIAP OriGene Custom Protein Services for XIAP
	GenScript Custom Purified and Recombinant Proteins Services for XIAP
	Novus Biologicals XIAP Proteins Novus Biologicals XIAP Lysates
	Sino Biological Recombinant Protein for XIAP
	Browse ProSpec Recombinant Proteins
	Uscn Proteins for XIAP

Gene Ontology (GO): 4 cellular component terms (GO ID links to tree view): About this table

GO ID	Qualified GO term	Evidence	PubMed IDs
GO:0005622	intracellular	--	--
GO:0005634	nucleus	IDA	--
GO:0005737	cytoplasm	IDA	--
GO:0005829	cytosol	TAS	--

XIAP for ontologies About GeneDecksing

XIAP Antibody Products:

	EMD Millipore Mono- and Polyclonal Antibodies for the study of XIAP
	R&D Systems Antibodies for XIAP
	Cell Signaling Technology (CST) Antibodies for XIAP
	OriGene Antibodies: XIAP OriGene Custom Antibody Services for XIAP
	GenScript Custom Superior Antibodies Services for XIAP
	Novus Biologicals XIAP Antibodies
	Abcam antibodies for XIAP
	Uscn Antibodies for XIAP
	ThermoFisher Antibody for XIAP

Assay Products for XIAP:

	Browse Kits and Assays available from EMD Millipore
	OriGene Custom Immunoassay Development Browse OriGene Fluorogenic Cell Assay Kits
	R&D Systems ELISAs for XIAP (see all)
	GenScript Custom Assay Services for XIAP
	Enzo Life Sciences assays for XIAP
	Uscn ELISAs and CLIAs for XIAP

(According to InterPro, ProtoNet, UniProtKB, and/or BLOCKS, Sets of similar genes according to GeneDecks)
About This Section

XIAP for domains About GeneDecksing

2 InterPro domains/families:

IPR001841 Znf_RING

IPR001370 BIR

Graphical View of Domain Structure for InterPro Entry P98170

ProtoNet protein and cluster: P98170

2 Blocks protein families:

IPB001370 Baculovirus inhibitor of apoptosis protein repeat (BIR)

IPB001841 Zn-finger

UniProtKB/Swiss-Prot: XIAP_HUMAN, P98170

Domain: The first BIR domain is involved in interaction with TAB1/MAP3K7IP1 and is important for dimerization. The

second BIR domain is sufficient to inhibit CASP3 and CASP7, while the third BIR is involved in CASP9 inhibition. The

interactions with DIABLO/SMAC and PRSS25 are mediated by the second and third BIR domains

Similarity: Belongs to the IAP family

Similarity: Contains 3 BIR repeats

Similarity: Contains 1 RING-type zinc finger

(According to ¹UniProtKB, Genatlas, LifeMap Discovery™, IUBMB, and/or ²DME, Human phenotypes from GenomeRNAi, Animal models from MGI Mar 06 2013,
bound targets from SABiosciences, miRNA Gene Targets from miRTarBase shRNA from OriGene, RNAi from EMD Millipore, siRNAs from OriGene, QIAGEN, microRNA from QIAGEN, Gene Editing from DNA2.0, Clones from EMD Millipore, OriGene, SwitchGear Genomics, GenScript, Sino Biological, DNA2.0, and Vector BioLabs, Cell Lines from GenScript, LifeMap BioReagents, In Situ Hybridization Assays from Advanced Cell Diagnostics, Ontologies according to Gene Ontology Consortium 01 Mar 2013 via Entrez Gene.)
About This Section

Function Summary: