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线粒体:在镰状细胞病中崭露头角的重要因素

Mitochondria: Emerging Consequential in Sickle Cell Disease.

作者信息

Akhter Mohammad S, Hamali Hassan A, Rashid Hina, Dobie Gasim, Madkhali Aymen M, Mobarki Abdullah A, Oldenburg Johannes, Biswas Arijit

机构信息

Department of Medical Laboratory Technology, College of Applied Medical Sciences, Jazan University, Jizan 45142, Saudi Arabia.

Department of Pharmacology and Toxicology, College of Pharmacy, Jazan University, Jizan 45142, Saudi Arabia.

出版信息

J Clin Med. 2023 Jan 18;12(3):765. doi: 10.3390/jcm12030765.

Abstract

Advanced mitochondrial multi-omics indicate a multi-facet involvement of mitochondria in the physiology of the cell, changing the perception of mitochondria from being just the energy-generating organelles to organelles that highly influence cell structure, function, signaling, and cell fate. This sets mitochondrial dysfunction in the centerstage of numerous acquired and genetic diseases. Sickle cell disease is also being increasingly associated with mitochondrial anomalies and the pathophysiology of sickle cell disease finds mitochondria at crucial intersections in the pathological cascade. Altered mitophagy, increased ROS, and mitochondrial DNA all contribute to the condition and its severity. Such mitochondrial aberrations lead to consequent mitochondrial retention in red blood cells in sickle cell diseases, increased oxidation in the cellular environment, inflammation, worsened vaso-occlusive crisis, etc. There are increasing studies indicating mitochondrial significance in sickle cell disease, consequently providing an opportunity to target it for improving the outcomes of treatment. Identification of the impaired mitochondrial attributes in sickle cell disease and their modulation by therapeutic interventions can impart a better management of the disease. This review aims to describe the mitochondria in the perspective of sicke cell disease so as to provide the reader an overview of the emerging mitochondrial stance in sickle cell disease.

摘要

先进的线粒体多组学研究表明,线粒体在细胞生理过程中具有多方面的作用,这改变了人们对线粒体的认知,即线粒体不再仅仅是产生能量的细胞器,而是对细胞结构、功能、信号传导和细胞命运有高度影响的细胞器。这使得线粒体功能障碍成为众多获得性疾病和遗传性疾病的核心问题。镰状细胞病也越来越多地与线粒体异常相关,镰状细胞病的病理生理学发现线粒体处于病理级联反应的关键节点。自噬改变、活性氧增加和线粒体DNA都与该病及其严重程度有关。这种线粒体异常导致镰状细胞病患者红细胞中线粒体滞留、细胞环境氧化增加、炎症、血管闭塞性危机恶化等。越来越多的研究表明线粒体在镰状细胞病中具有重要意义,从而为针对线粒体进行治疗以改善治疗效果提供了机会。识别镰状细胞病中线粒体受损的特征并通过治疗干预对其进行调节,可以更好地管理这种疾病。本综述旨在从镰状细胞病的角度描述线粒体,以便为读者提供镰状细胞病中线粒体新观点的概述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3e0c/9917941/16ad7e2f46de/jcm-12-00765-g001.jpg

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