The Wiskott-Aldrich syndrome protein is required for positive selection during T-cell lineage differentiation.
ATO
CRISPR/Cas9
INDEL
T-cell development
T-cell repertoire
Wiskott Aldrich syndrome
primary immunodeficiencies
Journal
Frontiers in immunology
ISSN: 1664-3224
Titre abrégé: Front Immunol
Pays: Switzerland
ID NLM: 101560960
Informations de publication
Date de publication:
2023
2023
Historique:
received:
16
03
2023
accepted:
15
05
2023
medline:
26
6
2023
pubmed:
23
6
2023
entrez:
23
6
2023
Statut:
epublish
Résumé
The Wiskott-Aldrich syndrome (WAS) is an X-linked primary immune deficiency caused by a mutation in the WAS gene. This leads to altered or absent WAS protein (WASp) expression and function resulting in thrombocytopenia, eczema, recurrent infections, and autoimmunity. In T cells, WASp is required for immune synapse formation. Patients with WAS show reduced numbers of peripheral blood T lymphocytes and an altered T-cell receptor repertoire.
Identifiants
pubmed: 37350958
doi: 10.3389/fimmu.2023.1188099
pmc: PMC10282776
doi:
Substances chimiques
Wiskott-Aldrich Syndrome Protein
0
Receptors, Antigen, T-Cell
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1188099Informations de copyright
Copyright © 2023 Pille, Avila, Sanchez, Goetgeluk, De Munter, Jansen, Billiet, Weening, Xue, Bonte, Ingels, De Cock, Pascal, Deseins, Kerre, Taghon, Leclercq, Vermijlen, Davis and Vandekerckhove.
Déclaration de conflit d'intérêts
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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