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- Title
The Heterogeneous Pathogenesis of Selective Immunoglobulin A Deficiency.
- Authors
Bagheri, Yasser; Sanaei, Roozbeh; Yazdani, Reza; Shekarabi, Mehdi; Falak, Reza; Mohammadi, Javad; Abolhassani, Hassan; Aghamohammadi, Asghar
- Abstract
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.
- Subjects
B cell differentiation; B cells; T cells; PLASMA cells; MAJOR histocompatibility complex
- Publication
International Archives of Allergy & Immunology, 2019, Vol 179, Issue 3, p231
- ISSN
1018-2438
- Publication type
Article
- DOI
10.1159/000499044