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Article

XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome.

Nature - 2 Nov 2006

Rigaud Stéphanie, Fondanèche Marie-Claude, Lambert Nathalie, Pasquier Benoit, Mateo Véronique, Soulas Pauline, Galicier Lionel, Le Deist Françoise, Rieux-Laucat Frédéric, Revy Patrick, Fischer Alain, de Saint Basile Geneviève, Latour Sylvain

Abstract excerpt

The homeostasis of the immune response requires tight regulation of the proliferation and apoptosis of activated lymphocytes. In humans, defects in immune homeostasis result in lymphoproliferation disorders including autoimmunity, haemophagocytic lymphohystiocytosis and lymphomas. The X-linked lymphoproliferative syndrome (XLP) is a rare, inherited immunodeficiency that is characterized by lymphohystiocytosis,...

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Source

Identifiers

PMID: 17080092
DOI: 10.1038/nature05257

Genes

XIAP
TNF

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome.PMID 17080092Read ↗

Regulation of Cell Death and Immunity by XIAP.Semantic proximity Read ↗

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease.Semantic proximity Read ↗

X-linked inhibitor of apoptosis (XIAP) deficiency: the spectrum of presenting manifestations beyond hemophagocytic lymphohistiocytosis.Semantic proximity Read ↗

X-linked lymphoproliferative syndromes and related autosomal recessive disorders.Semantic proximity Read ↗

A mutation in X-linked inhibitor of apoptosis (G466X) leads to memory inflation of Epstein-Barr virus-specific T cells.Semantic proximity Read ↗

Clinical and genetic characteristics of XIAP deficiency in Japan.Semantic proximity Read ↗

Novel XIAP mutation causing enhanced spontaneous apoptosis and disturbed NOD2 signalling in a patient with atypical adult-onset Crohn's disease.Semantic proximity Read ↗

SAP and XIAP deficiency in hemophagocytic lymphohistiocytosisSemantic proximity Read ↗

Using flow cytometry to screen patients for X-linked lymphoproliferative disease due to SAP deficiency and XIAP deficiency.Semantic proximity Read ↗

X-linked lymphoproliferative disease (XLP): a model of impaired anti-viral, anti-tumor and humoral immune responses.Semantic proximity Read ↗

X-linked lymphoproliferative syndromes: brothers or distant cousins?Semantic proximity Read ↗

Phenotype, genotype, treatment, and survival outcomes in patients with X-linked inhibitor of apoptosis deficiency.Semantic proximity Read ↗

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