Chronic Granulomatous disease: Lessons in Cell Biology From Monogenic Immunodeficiency.

IF 3.4 3区医学 Q3 IMMUNOLOGY

Clinical and experimental immunology Pub Date : 2025-05-15 DOI:10.1093/cei/uxaf031

Paige M Mortimer, Shuli Svetitsky, David C Thomas

引用次数: 0

Abstract

Reactive oxygen species (ROS) are produced in immune cells by the phagocyte NADPH oxidase (NOX2) system that carries out coordinated transfer of electrons to molecular oxygen. The importance of the system in host defence and immunoregulation is underlined by chronic granulomatous disease (CGD), a severe monogenic immunodeficiency caused by mutations in genes encoding individual components of NOX2. CGD also leads to inflammatory manifestations due to the regulatory role of ROS in multiple signalling processes. We describe the system in detail, from its discovery to our current understanding of the oxidase. We also describe CGD and illustrate how recent insights into this disease shed light on physiology.

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慢性肉芽肿病：从单基因免疫缺陷细胞生物学的教训。

活性氧（ROS）在免疫细胞中由吞噬细胞NADPH氧化酶（NOX2）系统产生，该系统将电子协调转移到分子氧中。慢性肉芽肿病（CGD）强调了该系统在宿主防御和免疫调节中的重要性，CGD是一种由编码NOX2单个组分的基因突变引起的严重单基因免疫缺陷。由于ROS在多个信号过程中的调节作用，CGD也会导致炎症表现。我们详细描述了这个系统，从它的发现到我们目前对氧化酶的理解。我们还描述了CGD，并说明了最近对这种疾病的见解如何阐明了生理学。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Clinical and experimental immunology 医学-免疫学

CiteScore

8.40

自引率

2.20%

发文量

101

审稿时长

3-8 weeks

期刊介绍： Clinical & Experimental Immunology (established in 1966) is an authoritative international journal publishing high-quality research studies in translational and clinical immunology that have the potential to transform our understanding of the immunopathology of human disease and/or change clinical practice. The journal is focused on translational and clinical immunology and is among the foremost journals in this field, attracting high-quality papers from across the world. Translation is viewed as a process of applying ideas, insights and discoveries generated through scientific studies to the treatment, prevention or diagnosis of human disease. Clinical immunology has evolved as a field to encompass the application of state-of-the-art technologies such as next-generation sequencing, metagenomics and high-dimensional phenotyping to understand mechanisms that govern the outcomes of clinical trials.