Mitochondrial ATP synthase: architecture, function and pathology

被引：437

作者：

Jonckheere, An I. ^{[1
]}

Smeitink, Jan A. M. ^{[1
]}

Rodenburg, Richard J. T. ^{[1
]}

机构：

[1] Radboud Univ Nijmegen, Dept Pediat, Nijmegen Ctr Mitochondrial Disorders, Lab Genet Endocrine & Metab Disorders 656,Med Ctr, NL-6500 HB Nijmegen, Netherlands

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 2012年 / 35卷 / 02期

关键词：

BLUE NATIVE ELECTROPHORESIS; LEIGH-SYNDROME; RESPIRATORY-CHAIN; T8993G MUTATION; OXIDATIVE-PHOSPHORYLATION; INHIBITOR PROTEIN; F1F0-ATP SYNTHASE; T9176C MUTATION; DNA MUTATION; SUPRAMOLECULAR ORGANIZATION;

D O I：

10.1007/s10545-011-9382-9

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Human mitochondrial (mt) ATP synthase, or complex V consists of two functional domains: F-1, situated in the mitochondrial matrix, and F-o, located in the inner mitochondrial membrane. Complex V uses the energy created by the proton electrochemical gradient to phosphorylate ADP to ATP. This review covers the architecture, function and assembly of complex V. The role of complex V di-and oligomerization and its relation with mitochondrial morphology is discussed. Finally, pathology related to complex V deficiency and current therapeutic strategies are highlighted. Despite the huge progress in this research field over the past decades, questions remain to be answered regarding the structure of subunits, the function of the rotary nanomotor at a molecular level, and the human complex V assembly process. The elucidation of more nuclear genetic defects will guide physio(patho)logical studies, paving the way for future therapeutic interventions.

引用

页码：211 / 225

页数：15

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