PMID- 36756122 OWN - NLM STAT- MEDLINE DCOM- 20240208 LR - 20240208 IS - 1664-3224 (Electronic) IS - 1664-3224 (Linking) VI - 14 DP - 2023 TI - Dendritic cells in inborn errors of immunity. PG - 1080129 LID - 10.3389/fimmu.2023.1080129 [doi] LID - 1080129 AB - 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. CI - Copyright (c) 2023 Gupta and Agrawal. FAU - Gupta, Sudhir AU - Gupta S AD - Division of Basic and Clinical Immunology, University of California, Irvine, CA, United States. FAU - Agrawal, Anshu AU - Agrawal A AD - Division of Basic and Clinical Immunology, University of California, Irvine, CA, United States. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20230123 PL - Switzerland TA - Front Immunol JT - Frontiers in immunology JID - 101560960 SB - IM MH - Humans MH - Autoimmunity MH - Dendritic Cells MH - Inflammation MH - *Primary Immunodeficiency Diseases MH - Immune Tolerance PMC - PMC9899832 OTO - NOTNLM OT - classical DCs OT - dendritic cells OT - inborn errors of immunity OT - monocyte derived DCs OT - myeloid DCs OT - plasmacytoid DCs OT - primary immunodeficiencies COIS- The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. EDAT- 2023/02/10 06:00 MHDA- 2023/02/11 06:00 PMCR- 2023/01/01 CRDT- 2023/02/09 02:22 PHST- 2022/10/25 00:00 [received] PHST- 2023/01/06 00:00 [accepted] PHST- 2023/02/09 02:22 [entrez] PHST- 2023/02/10 06:00 [pubmed] PHST- 2023/02/11 06:00 [medline] PHST- 2023/01/01 00:00 [pmc-release] AID - 10.3389/fimmu.2023.1080129 [doi] PST - epublish SO - Front Immunol. 2023 Jan 23;14:1080129. doi: 10.3389/fimmu.2023.1080129. eCollection 2023.