复发性嗜酸性粒细胞增多伴新的纯合子ARPC1B突变。

IF 3.9 3区 医学 Q2 MEDICINE, RESEARCH & EXPERIMENTAL
Gamze Sonmez, Baris Ulum, Ates Kutay Tenekeci, Canan Caka, Ali Şahin, Alp Kazancıoğlu, Begum Ozbek, İsmail Yaz, Saliha Esenboğa, Deniz Çağdaş
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引用次数: 0

摘要

细胞骨架网络失调是各种免疫缺陷和自身炎症的关键决定因素。本文综述了肌动蛋白重塑在疾病发病机制中的意义,重点介绍了Arp2/3复合体及其调控亚基肌动蛋白相关蛋白2/3复合体亚基1B (ARPC1B)。与ARPC1B缺陷相关的一系列细胞功能障碍,影响多种免疫细胞类型,已被阐明。该研究报告了一名复发性和持续性嗜酸性粒细胞增多的患者,这是由于纯合性ARPC1B突变与伴随的复合杂合性囊性纤维化跨膜传导调节因子(CFTR)基因突变引起的。采用ARPC1B抗体对患者和对照组外周血淋巴细胞进行染色。本例患者ARPC1B基因缺陷导致免疫荧光显微镜下ARPC1B基因缺失或低表达。细胞骨架缺陷和免疫表现之间复杂的相互作用强调了疾病表型的复杂性,需要进一步探索靶向治疗策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Recurrent eosinophilia with a novel homozygous ARPC1B mutation.

Cytoskeletal network dysregulation is a pivotal determinant in various immunodeficiencies and autoinflammatory conditions. This report reviews the significance of actin remodeling in disease pathogenesis, focusing on the Arp2/3 complex and its regulatory subunit actin related protein 2/3 complex subunit 1B (ARPC1B). A spectrum of cellular dysfunctions associated with ARPC1B deficiency, impacting diverse immune cell types, is elucidated. The study presents a patient featuring recurrent and persistent eosinophilia attributed to homozygous ARPC1B mutation alongside concomitant compound heterozygous cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations. We used ARPC1B antibody to stain the patient's peripheral blood lymphocytes and those of the control. The defect in the ARPC1B gene in the present patient caused absent/low expression by immunofluorescence microscopy. The intricate interplay between cytoskeletal defects and immunological manifestations underscores the complexity of disease phenotypes, warranting further exploration for targeted therapeutic strategies.

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来源期刊
Frontiers of Medicine
Frontiers of Medicine ONCOLOGYMEDICINE, RESEARCH & EXPERIMENTAL&-MEDICINE, RESEARCH & EXPERIMENTAL
CiteScore
18.30
自引率
0.00%
发文量
800
期刊介绍: Frontiers of Medicine is an international general medical journal sponsored by the Ministry of Education of China. The journal is jointly published by the Higher Education Press and Springer. Since the first issue of 2010, this journal has been indexed in PubMed/MEDLINE. Frontiers of Medicine is dedicated to publishing original research and review articles on the latest advances in clinical and basic medicine with a focus on epidemiology, traditional Chinese medicine, translational research, healthcare, public health and health policies.
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