Dendritic cell analysis in primary immunodeficiency.
Dendritic cell analysis in primary immunodeficiency.
复制标题
DOI：
10.1097/aci.0000000000000322
复制
发表时间：
2016-12
影响因子：
2.8
通讯作者：
Collin M
中科院分区：
文献类型：
--
作者：
Bigley V;Barge D;Collin M
树突状细胞是专门的抗原呈递细胞，通过识别抗原并将其呈递给 T 细胞，将先天免疫和适应性免疫联系起来。尽管树突状细胞的重要性已在许多动物模型中得到证明，但它们对人体免疫的贡献在体内仍然相对未经探索。鉴于树突状细胞在感染、自身免疫和恶性肿瘤中的核心作用，树突状细胞缺陷或功能障碍预计会产生临床后果。与 GATA 结合蛋白 2 (GATA2) 和干扰素调节因子 8 (IRF8) 突变相关的人类树突状细胞缺陷性疾病突出了树突状细胞和单核细胞在原发性免疫缺陷疾病中的重要性，并开始阐明它们在宿主防御和体内免疫调节中的非冗余作用。树突状细胞和单核细胞功能障碍对原发性免疫缺陷疾病表型发病机制的贡献变得越来越明显。然而，树突状细胞分析尚未成为原发性免疫缺陷疾病检查的常规部分。在临床实践中广泛采用树突状细胞/单核细胞筛查将有助于发现新型树突状细胞和单核细胞疾病，并增进我们对人类树突状细胞生物学在健康和疾病方面的理解。
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.