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Gene

XIAP

X-linked inhibitor of apoptosis

Summary

Cytogenetic location
Xq25
Coordinates
Chromosome X: 123,860,053–123,913,972 (GRCh38)
Exons
7 exons across 53.9 kb
Protein
497 amino acids
Inheritance
X-linked recessive
Also known as
hILP, ILP-1
Previous symbols
API3, BIRC4
MANE Select transcript
ENST00000371199.8NM_001167.4
Locus type
gene with protein product
  • Very high
  • Confident
  • Low
  • Very low

Reference genomeGRCh38

Positions on gene and variant pages follow this choice, in this browser.

Curated reports relevant to this gene

  • X-Linked Lymphoproliferative Disease and XMEN

    • X-linked lymphoproliferative diseaseX-linked recessive

      Severe EBV infection, HLH, low antibodies and lymphoma risk caused by changes in SH2D1A (XLP1) or XIAP (XLP2).

      • ClinGen rates the link definitive
      • 3 GenCC submitters rate it definitive or strong
      What knowing changes

      Stem cell transplantation can be curative for XLP1; diagnosis also guides HLH treatment, immunoglobulin replacement and testing of relatives.

Broader groupings

  • Other Inborn Errors of Immunity

    • Inborn error of immunity

      One of 114 genes linked to it

      Inherited disorders of the immune system caused by changes in single genes, affecting how the body fights infection or regulates inflammation.

      • ClinGen rates the link definitive
      • 3 GenCC submitters rate it definitive or strong
    • Immunodeficiency Disease

      One of 170 genes linked to it

      Disease in which there is a deficiency or defect in the mechanisms of immunity, either cellular or humoral.

      • ClinGen rates the link definitive
      • 3 GenCC submitters rate it definitive or strong
      What knowing changes

      HSCT for SCID and severe PIDs; immunoglobulin replacement (IVIG/SCIG) for antibody deficiencies; leniolisib (Joenja, FDA 2023) for APDS; gene therapy for ADA-SCID; abatacept for CTLA4/LRBA deficiency; prophylactic antibiotics; G-CSF for CGD

Inheritance

X-linked recessive
The change is on the X chromosome, so one copy affects males, who have one X, while females with one are usually carriers. A carrier mother passes it on to each child with a one-in-two chance.

How XIAP is laid out

  • Coding sequence
  • Untranslated region
  • Intron
  • Direction of transcription
  • Known disease-causing sites
  • Stretches short reads find hard
Chromosome X, with a bracket where XIAP sits. XIAP and 6 genes near it on chromosome X, from 123,387,013 to 124,387,012.Chromosome XXq25p22.33p22.32p22.31p22.2p22.13p22.12p22.11p21.3p21.2p21.1p11.4p11.3p11.23p11.22p11.21p11.1q11.1q11.2q12q13.1q13.2q13.3q21.1q21.2q21.31q21.32q21.33q22.1q22.2q22.3q23q24q25q26.1q26.2q26.3q27.1q27.2q27.3q28123.4 Mb123.6 Mb123.8 Mb124 Mb124.2 MbGRIA3: 123,184,023–123,490,915, forward strandGRIA3THOC2: 123,600,561–123,733,061, reverse strandTHOC2XIAP: 123,860,053–123,913,972, forward strandXIAPSTAG2: 123,960,212–124,422,664, forward strandSTAG2SH2D1A: 124,227,868–124,373,197, forward strandSH2D1ATEX13D: 124,246,249–124,336,863, forward strandTEX13DTENM1: 124,375,903–125,204,312, reverse strandTENM1Diagram of XIAP: 7 exons on the forward strand of chromosome XExon 2: 28 known disease-causing sitesExon 3: 5 known disease-causing sitesExon 4: 8 known disease-causing sitesExon 5: 1 known disease-causing siteExon 6: 3 known disease-causing sitesExon 7: 5 known disease-causing sitesExon 1: 123,860,053–123,860,293, 241 basesExon 2: 123,885,631–123,886,539, 909 basesExon 3: 123,888,619–123,888,718, 100 basesExon 4: 123,891,238–123,891,316, 79 basesExon 5: 123,892,731–123,892,773, 43 basesExon 6: 123,900,493–123,900,693, 201 basesExon 7: 123,906,988–123,913,972, 6,985 bases12345675′3′

Exons of the gene's MANE Select transcript (release v1.5), from NCBI and EMBL-EBI, on the GRCh38 reference genome.

Chromosome bands from UCSC's GRCh38 ideogram (2022-10-29).

Nearby genes from Ensembl release 116, on GRCh38.

Exons make up 8.6 kb of the gene's 53.9 kb; 1,494 of those bases code for the protein.

How a genome test reads XIAP

58

positions in XIAP where ClinVar classes a change as pathogenic or likely pathogenic

5 of them (8.6%) fall in regions short reads find hard.

  • Low mappability (3 sites)Regions where 100 bp or 250 bp reads cannot be placed uniquely.
  • Segmental duplications (5 sites)Copies of at least 1 kb sharing at least 90% identity with another part of the genome (paralogues, pseudogenes); reads may align to the wrong copy.

A whole genome covers all 53.9 kb of XIAP, introns included, not only the 8.6 kb of exons.

Where this comes from

  • HGNC: the gene's symbol, name, other symbols and location.
  • MANE Select v1.5, from NCBI and EMBL-EBI: the transcript, its exons and coding sequence.
  • GenCC and ClinGen, released 2026-10-16: which conditions the gene is linked to, and how strongly.
  • The ACMG secondary findings list, SF v3.2.
  • ClinVar and the GIAB stratifications: known disease-causing sites, and the regions short reads find hard.
  • The Human Phenotype Ontology, release 2026-09-01: the traits linked to the gene. Find out more at hpo.jax.org.
  • Data release 2026.10.16-c6eea6bf.

Cite this page

APA

Dante Labs. (n.d.). XIAP gene: X-linked inhibitor of apoptosis. Retrieved 17 October 2026, from https://dantelabs.com/genes/xiap

Vancouver

Dante Labs. XIAP gene: X-linked inhibitor of apoptosis [Internet]. [cited 17 October 2026]. Available from: https://dantelabs.com/genes/xiap

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