کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8716113 | 1587878 | 2018 | 11 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Impaired Specific Antifungal Immunity in CARD9-Deficient Patients with Phaeohyphomycosis
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کلمات کلیدی
موضوعات مرتبط
علوم پزشکی و سلامت
پزشکی و دندانپزشکی
امراض پوستی
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چکیده انگلیسی
Phaeohyphomycosis is a group of severe infections caused by dematiaceous fungi. We previously identified CARD9 deficiencies in four Chinese patients with phaeohyphomycosis caused by Phialophora verrucosa. In this study, we sought to identify the genetic and immunological mechanisms underlying rare dematiaceous fungal infections in three otherwise healthy patients with phaeohyphomycosis caused by Exophiala spinifera, Ochroconis musae, and Corynespora cassiicola. CARD9 sequencing in these patients showed one mutation (p.S23X) that, to our knowledge, has not been characterized and two previously characterized mutations (p.D274fsX60 and p.L64fsX59) that led to lack of CARD9 protein expression. Patient-derived CARD9-deficient cells showed a selective impairment of proinflammatory cytokine and chemokine production, NF-κB activation, and T helper type 22- and T helper type 17-associated responses upon fungus-specific stimulation, whereas phagocytosis and reactive oxygen species production were intact. Consistently, Card9-knockout mice were highly susceptible to phaeohyphomycosis and exhibited immune deficiencies similar to those of patients, including diminished NF-κB and p38 MAPK activation in local and in vitro functional studies. This work clarifies the association between inherited CARD9 deficiencies and phaeohyphomycosis, and furthers current knowledge on the spectrum and pathophysiology of diseases resulting from CARD9 deficiencies.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Investigative Dermatology - Volume 138, Issue 3, March 2018, Pages 607-617
Journal: Journal of Investigative Dermatology - Volume 138, Issue 3, March 2018, Pages 607-617
نویسندگان
Xiaowen Wang, Ruijun Zhang, Weiwei Wu, Yinggai Song, Zhe Wan, Wenling Han, Ruoyu Li,