title: Dendritic cell analysis in primary immunodeficiency.
abstract: Dendritic 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.
  
  
  
  Human 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.
  
  
  
  Widespread 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.
authors.affiliation: aHuman Dendritic Cell Laboratory, Institute of Cellular Medicine, Newcastle University bBlood Sciences Flow Cytometry Laboratory, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
authors.forename: Venetia
authors.initials: V
authors.lastname: Bigley
authors.forename: Dawn
authors.initials: D
authors.lastname: Barge
authors.forename: Matthew
authors.initials: M
authors.lastname: Collin
mesh_terms: D056704:Adaptive Immunity; D000818:Animals; D017951:Antigen Presentation; D015551:Autoimmunity; D003713:Dendritic Cells / Q000502:physiology*; D050989:GATA2 Transcription Factor / Q000235:genetics*; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000276:immunology*; D050835:Interferon Regulatory Factors / Q000235:genetics*; D009000:Monocytes / Q000502:physiology*; D009154:Mutation / Q000235:genetics; D013601:T-Lymphocytes / Q000276:immunology; D000099185:Interferon Regulatory Factor-8
chemical_list: D050989:GATA2 Transcription Factor; D050835:Interferon Regulatory Factors; D000099185:Interferon Regulatory Factor-8; C494711:GATA2 protein, human