Oxidative Phosphorylation: Synthesis of Mitochondrially Encoded Proteins and Assembly of Individual Structural Subunits into Functional Holoenzyme Complexes

Oxidative Phosphorylation: Synthesis of Mitochondrially Encoded Proteins and Assembly of Individual Structural Subunits into Functional Holoenzyme Complexes
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DOI:
10.1007/978-1-59745-521-3_10
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发表时间:
2009-01-01
期刊:
MITOCHONDRIAL DNA: METHODS AND PROTOCOLS
影响因子:
--
通讯作者:
Sasarman, Florin
Sasarman, Florin
中科院分区:
其他
文献类型:
--
作者:
Leary, Scot C.;Sasarman, Florin

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高等真核生物中各种细胞过程消耗的大部分 ATP 通常是由嵌入线粒体内膜内的五种多聚蛋白复合物 (I-V) 产生的,这一过程称为氧化磷酸化 (OXPHOS)。因此,在大多数生理条件下能量稳态的维持取决于 OXPHOS 满足细胞生物能量需求变化的能力,长期无法做到这一点是人类疾病的常见原因。除复合物 II 外,OXPHOS 复合物的结构亚基均由核基因组和线粒体基因组编码。两个基因组的物理分离需要 13 种线粒体编码多肽的表达与相关核编码伴侣的表达相协调,以便组装功能性全酶复合物。复杂的生物发生是一个高度有序的过程,并且已经确定了在各个 OXPHOS 复合物组装的不同阶段发挥作用的几种核编码因子。
The bulk of ATP consumed by various Cellular processes in higher eukaryotes is normally produced by five multimeric protein complexes (I-V) embedded within the inner mitochondrial membrane, in a process known as oxidative phosphorylation (OXPHOS). Maintenance of energy homeostasis under most physiological conditions is therefore contingent upon the ability of OXPHOS to meet cellular changes in bioenergetic demand, with a chronic failure to do so being a frequent cause of human disease. With the exception of Complex II, the structural subunits of OXPHOS complexes are encoded by both the nuclear and the mitochondrial genomes. The physical separation of the two genomes necessitates that the expression of the 13 mitochondrially encoded polypeptides be co-ordinated with that of relevant nuclear-encoded partners in order to assemble functional holoenzyme complexes. Complex biogenesis is a highly ordered process, and several nuclear-encoded factors that function at distinct stages in the assembly of individual OXPHOS complexes have been identified.