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2016 ; 16
(6
): 530-540
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Dendritic cell analysis in primary immunodeficiency
#MMPMID27755182
Bigley V
; Barge D
; Collin M
Curr Opin Allergy Clin Immunol
2016[Dec]; 16
(6
): 530-540
PMID27755182
show ga
PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.