The mitochondrial oxidative phosphorylation (OXPHOS) system, responsible for aerobic energy production in nearly all cells in our body, comprises four enzyme complexes (Complexes I-IV) that make up the mitochondrial respiratory chain itself, and the ATP synthase complex (Complex V), which uses the energy generated by electron transport along the respiratory chain to produce ATP. The subunits of the five enzyme complexes are encoded in both the nuclear and mitochondrial genomes; of the 82 structural subunits, 13 are encoded in the mitochondrial genome (mtDNA), which also codes for the two rRNAs and 16 tRNAs necessary for their translation. OXPHOS deficiencies are an important cause of a wide range of neurological, neuromuscular, cardiac and endocrinedisorders, and even some cancers (Koopmaii ct al.. 2012; Nunnari and Suonialamen. 2012; Schon and Przcdborski. 2011; Ylikallio and Suomalainen, 2012), the minimum prevalence of which is estimated at -1:5000 (Chinncry. 2001). The molecular basis for this extraordinary range of clinical phenotypes remains an enduring mystery.
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机译：在本体中的几乎所有细胞中，负责有氧能量产生的线粒体氧化磷酸化（毒物）系统，包括四种酶复合物（复合物I-IV），其构成线粒体呼吸链本身，以及ATP合酶络合物（复杂V） ，它使用沿着呼吸链产生的电子传输产生的能量以产生ATP。五种酶复合物的亚基在核和线粒体基因组中编码;在82个结构亚基中，13在线粒体基因组（MTDNA）中编码，其还针对其翻译所需的两个RRNA和16个TRNA。毒物缺陷是广泛的神经，神经肌肉，心脏和内华松和内分泌等的重要原因，甚至是一些癌症（Koopmaii Ct al .. 2012; Nunnari和Suonialamen。2012; Schon和Przcdborski。2011; Ylikallio和Suomalainen，2012），最小患病率估计为-1：5000（Chinncry。2001）。这种非凡的临床表型范围的分子基础仍然是一种持久的谜。
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