A multimorphic mutation in IRF4 causes human autosomal dominant combined immunodeficiency.


Journal

Science immunology
ISSN: 2470-9468
Titre abrégé: Sci Immunol
Pays: United States
ID NLM: 101688624

Informations de publication

Date de publication:
20 01 2023
Historique:
entrez: 20 1 2023
pubmed: 21 1 2023
medline: 25 1 2023
Statut: ppublish

Résumé

Interferon regulatory factor 4 (IRF4) is a transcription factor (TF) and key regulator of immune cell development and function. We report a recurrent heterozygous mutation in IRF4, p.T95R, causing an autosomal dominant combined immunodeficiency (CID) in seven patients from six unrelated families. The patients exhibited profound susceptibility to opportunistic infections, notably

Identifiants

pubmed: 36662884
doi: 10.1126/sciimmunol.ade7953
doi:

Substances chimiques

Interferon Regulatory Factors 0
DNA 9007-49-2

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

eade7953

Auteurs

Consortium members and affiliations are located at the end of this paper. Individual contributions are listed in the Acknowledgements.

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Classifications MeSH