期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
卷 1793, 期 1, 页码 200-211出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbamcr.2008.05.028
关键词
Oxidative phosphorylation; OXPHOS; Mitochondrial respiratory chain; Respiratory chain complex; Assembly; Mitochondrial disorder; Mitochondrial DNA; Mitochondrial inner membrane; Electron transfer chain
资金
- Pierfranco and Luisa Mariani Foundation
- Fondazione Telethon-Italy [GGP07019]
- Italian Ministry of University and Research [RBLA038RMA]
- Italian Ministry of Health [RF2006 ex 56/05121]
- Marie Curie Intra-European Fellowship [040140-MAD]
- MITOCIRCLE
- European Union [LSHM-CT-2004-503116]
Assembly of the oxidative phosphorylation (OXPHOS) system in the mitochondrial inner membrane is an intricate process in which many factors must interact. The OXPHOS system is composed of four respiratory chain complexes, which are responsible for electron transport and generation of the proton gradient in the mitochondrial intermembrane space, and of the ATP synthase that uses this proton gradient to produce ATP. Mitochondrial human disorders are caused by dysfunction of the OXPHOS system, and many of them are associated with altered assembly of one or more components of the OXPHOS system. The study of assembly defects in patients has been useful in unraveling and/or gaining a complete understanding of the processes by which these large multimeric complexes are formed. We review here current knowledge of the biogenesis of OXPHOS complexes based on investigation of the corresponding disorders. (C) 2008 Elsevier B.V. All rights reserved.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据