Primary and secondary defects of the thymus.
AIRE
DiGeorge syndrome
FOXN1
T-cell reconstitution
athymia
immune restoration
immunodeficiency
immunosenescence
thymic atrophy
thymic epithelial cells
thymic involution
thymic regeneration
thymopoiesis
thymus
thymus transplantation
Journal
Immunological reviews
ISSN: 1600-065X
Titre abrégé: Immunol Rev
Pays: England
ID NLM: 7702118
Informations de publication
Date de publication:
Mar 2024
Mar 2024
Historique:
pubmed:
17
1
2024
medline:
17
1
2024
entrez:
16
1
2024
Statut:
ppublish
Résumé
The thymus is the primary site of T-cell development, enabling generation, and selection of a diverse repertoire of T cells that recognize non-self, whilst remaining tolerant to self- antigens. Severe congenital disorders of thymic development (athymia) can be fatal if left untreated due to infections, and thymic tissue implantation is the only cure. While newborn screening for severe combined immune deficiency has allowed improved detection at birth of congenital athymia, thymic disorders acquired later in life are still underrecognized and assessing the quality of thymic function in such conditions remains a challenge. The thymus is sensitive to injury elicited from a variety of endogenous and exogenous factors, and its self-renewal capacity decreases with age. Secondary and age-related forms of thymic dysfunction may lead to an increased risk of infections, malignancy, and autoimmunity. Promising results have been obtained in preclinical models and clinical trials upon administration of soluble factors promoting thymic regeneration, but to date no therapy is approved for clinical use. In this review we provide a background on thymus development, function, and age-related involution. We discuss disease mechanisms, diagnostic, and therapeutic approaches for primary and secondary thymic defects.
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
178-211Subventions
Organisme : Division of Intramural Research Programs of the National Institute of Allergy and Infectious Diseases and of the National Institute of Dental and Craniofacial Research, National Institutes of Health
ID : AI001270
Informations de copyright
Published 2024. This article is a U.S. Government work and is in the public domain in the USA.
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