The Heterogeneous Pathogenesis of Selective Immunoglobulin A Deficiency.
B cells defect
Cytokine defect
Genetic defect
Primary immunodeficiency
Selective immunoglobulin A deficiency
T cells defect
Journal
International archives of allergy and immunology
ISSN: 1423-0097
Titre abrégé: Int Arch Allergy Immunol
Pays: Switzerland
ID NLM: 9211652
Informations de publication
Date de publication:
2019
2019
Historique:
received:
04
01
2019
accepted:
18
02
2019
pubmed:
16
5
2019
medline:
14
8
2019
entrez:
16
5
2019
Statut:
ppublish
Résumé
Selective immunoglobulin A deficiency (SIgAD) is the most prevalent type of primary immunodeficiency disorder. The phenotypic feature of SIgAD is related to a defect in B lymphocyte differentiation into plasma cell-producing immunoglobulin A (IgA). In this review, we summarize the recent advances in this regard. Genetic (including major histocompatibility complex [MHC] and non-MHC genes), immunologic (including B and T lymphocyte subsets abnormality), cytokines/chemokines and their related receptors, apoptosis and microbiota defects are reviewed. The mechanisms leading to SIgAD are most likely multifactorial and it can be speculated that several pathways controlling B cells functions or regulating epigenetic of the IGHA gene encoding constant region of IgA heavy chain and long-term survival of IgA switched memory B cells and plasma cells may be defective in different SIgAD patients.
Identifiants
pubmed: 31091523
pii: 000499044
doi: 10.1159/000499044
doi:
Substances chimiques
Cytokines
0
Receptors, Immunologic
0
Types de publication
Journal Article
Review
Langues
eng
Pagination
231-246Informations de copyright
© 2019 S. Karger AG, Basel.