Abstract
Purpose of reviewDendritic cells are specialized antigen-presenting cells which link innate and adaptive immunity, through recognition and presentation of antigen to T cells. Although the importance of dendritic cells has been demonstrated in many animal models, their contribution to human immunity remains relatively unexplored in vivo.Given their central role in infection, autoimmunity, and malignancy, dendritic cell deficiency or dysfunction would be expected to have clinical consequences.Recent findingsHuman dendritic cell deficiency disorders, related to GATA binding protein 2 (GATA2) and interferon regulatory factor 8 (IRF8) mutations, have highlighted the importance of dendritic cells and monocytes in primary immunodeficiency diseases and begun to shed light on their nonredundant roles in host defense and immune regulation in vivo. The contribution of dendritic cell and monocyte dysfunction to the pathogenesis of primary immunodeficiency disease phenotypes is becoming increasingly apparent. However, dendritic cell analysis is not yet a routine part of primary immunodeficiency disease workup.SummaryWidespread uptake of dendritic cell/monocyte screening in clinical practice will facilitate the discovery of novel dendritic cell and monocyte disorders as well as advancing our understanding of human dendritic cell biology in health and disease.
Highlights
Much of our knowledge of dendritic cell biology is inferred from mouse models and human in-vitro systems, which may not reflect the steady state and provide a limited understanding of host defense in the intact human
We explore what is known about dendritic cells and monocytes in primary immunodeficiency disease (PID), highlight the recently described dendritic cell deficiency syndromes related to GATA binding protein 2 (GATA2) and interferon regulatory factor 8 (IRF8) mutations, suggest a practical solution to dendritic cell analysis in clinical practice, and speculate how best to further our understanding of dendritic cells in PID and immunity in general
Analysis of the monocyte/dendritic cell compartment revealed a subtle defect in CD1c expression on cDC2s, which is at odds with the relative dependence of cDC1 and plasmacytoid dendritic cells (pDCs) on IRF8 in mouse
Summary
Much of our knowledge of dendritic cell biology is inferred from mouse models and human in-vitro systems, which may not reflect the steady state and provide a limited understanding of host defense in the intact human. Populations of peripheral tissue dendritic cells, upon pathogen detection, become activated and migrate to T cell areas of draining lymph nodes (LNs) where they stimulate antigenspecific T cell responses to initiate immunity or tolerance. AHuman Dendritic Cell Laboratory, Institute of Cellular Medicine, Newcastle University and bBlood Sciences Flow Cytometry Laboratory, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callMore From: Current Opinion in Allergy & Clinical Immunology
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
