Marín-García José, Goldenthal Michael J
The Molecular Cardiology and Neuromuscular Institute. Highland Park, NJ 08904, USA.
Rev Esp Cardiol. 2002 Dec;55(12):1293-310. doi: 10.1016/s0300-8932(02)76802-4.
The heart is highly dependent for its function on oxidative energy generated in mitochondria, primarily by fatty acid beta-oxidation, respiratory electron chain and oxidative phosphorylation. Defects in mitochondrial structure and function have been found in association with cardiovascular diseases such as dilated and hypertrophy cardiomyopathy, cardiac conduction defects and sudden death, ischemic and alcoholic cardiomyopathy, as well as myocarditis. While a subset of these mitochondrial abnormalities have a defined genetic basis (e.g. mitochondrial DNA changes leading to oxidative phosphorylation dysfunction,fatty acid beta-oxidation defects due to specific nuclear DNA mutations), other abnormalities appear to be due to a more sporadic or environmental cardiotoxic insult or have not yet been characterized.This review focuses on abnormalities in mitochondrial bioenergetic function and mitochondrial DNA defects associated with cardiovascular diseases, their significance in cardiac pathogenesis as well as on the available diagnostic and therapeutic options. A concise background concerning mitochondrial biogenesis and bioenergetic pathways during cardiac growth,development and aging will also be provided.
心脏功能高度依赖线粒体产生的氧化能量，主要通过脂肪酸β氧化、呼吸电子链和氧化磷酸化来实现。线粒体结构和功能的缺陷已被发现与心血管疾病相关，如扩张型和肥厚型心肌病、心脏传导缺陷和猝死、缺血性和酒精性心肌病以及心肌炎。虽然这些线粒体异常中的一部分具有明确的遗传基础（例如线粒体DNA变化导致氧化磷酸化功能障碍、特定核DNA突变引起的脂肪酸β氧化缺陷），但其他异常似乎是由于更散发性或环境性心脏毒性损伤所致，或者尚未得到明确特征描述。本综述重点关注与心血管疾病相关的线粒体生物能量功能异常和线粒体DNA缺陷、它们在心脏发病机制中的意义以及现有的诊断和治疗选择。还将提供关于心脏生长、发育和衰老过程中线粒体生物发生和生物能量途径的简要背景知识。
