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The Heterogeneous P...
The Heterogeneous Pathogenesis of Selective Immunoglobulin A Deficiency
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Bagheri, Y (författare)
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Sanaei, R (författare)
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Yazdani, R (författare)
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Shekarabi, M (författare)
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Falak, R (författare)
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Mohammadi, J (författare)
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- Abolhassani, H (författare)
- Karolinska Institutet
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Aghamohammadi, A (författare)
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(creator_code:org_t)
- 2019-05-15
- 2019
- Engelska.
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Ingår i: International archives of allergy and immunology. - : S. Karger AG. - 1423-0097 .- 1018-2438. ; 179:3, s. 231-245
- Relaterad länk:
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https://www.karger.c...
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http://kipublication...
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https://doi.org/10.1...
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Abstract
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- 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 <i>IGHA</i> 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.
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