Institute of Biochemistry and Clinical Biochemistry, Università Cattolica del Sacro Cuore, Rome, Italy.
Fondazione Policlinico Universitario A. Gemelli - IRCCS, Rome, Italy.
Adv Exp Med Biol. 2019;1158:71-82. doi: 10.1007/978-981-13-8367-0_5.
Proteins oxidation by reactive species is implicated in the aetiology or progression of a panoply of disorders and diseases such as neurodegenerative disorders. It is becoming increasingly evident that redox imbalance in the brain mediates neurodegeneration. Free radicals, as reactive species of oxygen (ROS) but also reactive nitrogen species (RNS) and reactive sulfur species (RSS), are generated in vivo from several sources. Within the cell the mitochondria represent the main source of ROS and mitochondrial dysfunction is both the major contributor to oxidative stress (OS) as well its major consequence.To date there are no doubts that a condition of OS added to other factors as mitochondrial damage in mtDNA or mitochondrial respiratory chain, may contribute to trigger or amplify mechanisms leading to neurodegenerative disorders.In this chapter, we aim at illustrate the molecular interplay occurring between mitochondria and OS focusing on Amyotrophic Lateral Sclerosis, describing a phenotypic reprogramming mechanism of mitochondria in complex neurological disorder.
活性物质引起的蛋白质氧化与多种疾病的发病机制或进展有关，如神经退行性疾病。越来越明显的是，大脑中的氧化还原失衡介导神经退行性变。自由基作为活性氧（ROS），但也有活性氮（RNS）和活性硫（RSS），是由多个来源在体内产生的。在细胞内，线粒体是 ROS 的主要来源，线粒体功能障碍既是氧化应激（OS）的主要贡献者，也是其主要后果。迄今为止，毫无疑问，OS 状态加上其他因素，如 mtDNA 或线粒体呼吸链中的线粒体损伤，可能有助于引发或放大导致神经退行性疾病的机制。在本章中，我们旨在阐明线粒体与 OS 之间发生的分子相互作用，重点介绍肌萎缩侧索硬化症，描述复杂神经疾病中线粒体的表型重编程机制。
