期刊
FRONTIERS IN IMMUNOLOGY
卷 14, 期 -, 页码 -出版社
FRONTIERS MEDIA SA
DOI: 10.3389/fimmu.2023.1080129
关键词
dendritic cells; inborn errors of immunity; primary immunodeficiencies; plasmacytoid DCs; myeloid DCs; classical DCs; monocyte derived DCs
类别
In this study, the authors reviewed the biology of dendritic cells (DCs) in inborn errors of immunity (IEI) and discussed the role of DCs in clinical and immunological manifestations. They focused on severe immunodeficiency diseases, antibody deficiencies, combined immunodeficiency with associated and syndromic features, disorders of synapse formation, and disorders of immune regulation.
Dendritic cells (DCs) are crucial cells for initiating and maintaining immune response. They play critical role in homeostasis, inflammation, and autoimmunity. A number of molecules regulate their functions including synapse formation, migration, immunity, and induction of tolerance. A number of IEI are characterized by mutations in genes encoding several of these molecules resulting in immunodeficiency, inflammation, and autoimmunity in IEI. Currently, there are 465 Inborn errors of immunity (IEI) that have been grouped in 10 different categories. However, comprehensive studies of DCs have been reported in only few IEI. Here we have reviewed biology of DCs in IEI classified according to recently published IUIS classification. We have reviewed DCs in selected IEI in each group category and discussed in depth changes in DCs where significant data are available regarding role of DCs in clinical and immunological manifestations. These include severe immunodeficiency diseases, antibody deficiencies, combined immunodeficiency with associated and syndromic features, especially disorders of synapse formation, and disorders of immune regulation.
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