线粒体呼吸链复合体I：结构、功能及在人类疾病中的意义
Mitochondrial respiratory complex I: structure, function and implication in human diseases.
作者信息
Sharma Lokendra K, Lu Jianxin, Bai Yidong
机构信息
Department of Cellular and Structural Biology, University of Texas Health Sciences Center at San Antonio, San Antonio, TX 78229, USA.
出版信息
Curr Med Chem. 2009;16(10):1266-77. doi: 10.2174/092986709787846578.
Abstract
Mitochondria are ubiquitous organelles in eukaryotic cells whose primary function is to generate energy supplies in the form of ATP through oxidative phosphorylation. As the entry point for most electrons into the respiratory chain, NADH:ubiquinone oxidoreductase, or complex I, is the largest and least understood component of the mitochondrial oxidative phosphorylation system. Substantial progress has been made in recent years in understanding its subunit composition, its assembly, the interaction among complex I and other respiratory components, and its role in oxidative stress and apoptosis. This review provides an updated overview of the structure of complex I, as well as its cellular functions, and discusses the implication of complex I dysfunction in various human diseases.
摘要
线粒体是真核细胞中普遍存在的细胞器，其主要功能是通过氧化磷酸化产生ATP形式的能量供应。作为大多数电子进入呼吸链的入口点，NADH：泛醌氧化还原酶，即复合体I，是线粒体氧化磷酸化系统中最大且了解最少的组分。近年来，在了解其亚基组成、组装、复合体I与其他呼吸组分之间的相互作用以及其在氧化应激和细胞凋亡中的作用方面取得了重大进展。本综述提供了复合体I结构及其细胞功能的最新概述，并讨论了复合体I功能障碍在各种人类疾病中的影响。
相似文献
1
Mitochondrial respiratory complex I: structure, function and implication in human diseases.
Curr Med Chem. 2009;16(10):1266-77. doi: 10.2174/092986709787846578.
2
Molecular mechanisms of superoxide production by the mitochondrial respiratory chain.
Adv Exp Med Biol. 2012;748:145-69. doi: 10.1007/978-1-4614-3573-0_6.
3
Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation.
Antioxid Redox Signal. 2010 Jun 15;12(12):1431-70. doi: 10.1089/ars.2009.2743.
4
Post-Translational Oxidative Modifications of Mitochondrial Complex I (NADH: Ubiquinone Oxidoreductase): Implications for Pathogenesis and Therapeutics in Human Diseases.
J Alzheimers Dis. 2017;60(s1):S69-S86. doi: 10.3233/JAD-170117.
5
Structure and function of mitochondrial complex I.
Biochim Biophys Acta. 2016 Jul;1857(7):902-14. doi: 10.1016/j.bbabio.2016.02.013. Epub 2016 Feb 24.
6
Mitochondrial respiratory chain complexes: apoptosis sensors mutated in cancer?
Oncogene. 2011 Sep 22;30(38):3985-4003. doi: 10.1038/onc.2011.167. Epub 2011 May 30.
7
Mitochondrial Complex I: structure, function, and implications in neurodegeneration.
Ital J Biochem. 2006 Sep-Dec;55(3-4):232-53.
8
Complex I function in mitochondrial supercomplexes.
Biochim Biophys Acta. 2016 Jul;1857(7):991-1000. doi: 10.1016/j.bbabio.2016.01.013. Epub 2016 Jan 25.
9
A new insight into the molecular hydrogen effect on coenzyme Q and mitochondrial function of rats.
Can J Physiol Pharmacol. 2020 Jan;98(1):29-34. doi: 10.1139/cjpp-2019-0281. Epub 2019 Sep 19.
10
In vitro characterization of mitochondrial function and structure in rat and human cells with a deficiency of the NADH: ubiquinone oxidoreductase Ndufc2 subunit.
Hum Mol Genet. 2017 Dec 1;26(23):4541-4555. doi: 10.1093/hmg/ddx333.
引用本文的文献
1
NDUFS1 upregulates ENaCα by NAD+ to promote alveolar fluid clearance in acute lung injury.
Int J Med Sci. 2025 Jul 28;22(13):3477-3489. doi: 10.7150/ijms.112248. eCollection 2025.
2
The Loss of Complex I in Renal Oncocytoma Is Associated with Defective Mitophagy Due to Lysosomal Dysfunction.
Int J Mol Sci. 2025 Aug 7;26(15):7654. doi: 10.3390/ijms26157654.
3
Sigma-1 Receptor Promotes Glycolysis in Neuronal Systems by Suppressing GRIM19.
bioRxiv. 2025 Jul 31:2025.07.28.667250. doi: 10.1101/2025.07.28.667250.
4
The metabolic costs of meiotic drive.
Proc Biol Sci. 2025 Jul;292(2050):20250779. doi: 10.1098/rspb.2025.0779. Epub 2025 Jul 2.
5
Pneumococcal HO Reshapes Mitochondrial Function and Reprograms Host Cell Metabolism.
bioRxiv. 2025 May 22:2025.05.22.655446. doi: 10.1101/2025.05.22.655446.
6
A Comprehensive Review of Mitochondrial Complex I During Mammalian Oocyte Maturation.
Genesis. 2025 Jun;63(3):e70017. doi: 10.1002/dvg.70017.
7
Rational engineering unlocks the therapeutic potential of WHP1: A revolutionary peptide poised to advance wound healing.
PLoS One. 2025 May 14;20(5):e0323363. doi: 10.1371/journal.pone.0323363. eCollection 2025.
8
Innovations in Drug Discovery for Sickle Cell Disease Targeting Oxidative Stress and NRF2 Activation-A Short Review.
Int J Mol Sci. 2025 Apr 28;26(9):4192. doi: 10.3390/ijms26094192.
9
Restoration of pp60 Re-Establishes Electron Transport Chain Complex I Activity in Pulmonary Hypertensive Endothelial Cells.
Int J Mol Sci. 2025 Apr 17;26(8):3815. doi: 10.3390/ijms26083815.
10
Effects of the Quinone Analog Ubiquinone-5 on Murine Mitochondria and Hypnosis.
Anesthesiology. 2025 Sep 1;143(3):641-660. doi: 10.1097/ALN.0000000000005549. Epub 2025 May 2.
本文引用的文献
1
A heteroplasmic, not homoplasmic, mitochondrial DNA mutation promotes tumorigenesis via alteration in reactive oxygen species generation and apoptosis.
Hum Mol Genet. 2009 May 1;18(9):1578-89. doi: 10.1093/hmg/ddp069. Epub 2009 Feb 10.
2
Mitochondrial DNA mutations in the D-loop region may not be frequent in cervical cancer: a discussion on pitfalls in mitochondrial DNA studies.
J Cancer Res Clin Oncol. 2009 Apr;135(4):649-51. doi: 10.1007/s00432-008-0542-8. Epub 2009 Jan 14.
3
Mutation of C20orf7 disrupts complex I assembly and causes lethal neonatal mitochondrial disease.
Am J Hum Genet. 2008 Oct;83(4):468-78. doi: 10.1016/j.ajhg.2008.09.009.
4
The production of reactive oxygen species by complex I.
Biochem Soc Trans. 2008 Oct;36(Pt 5):976-80. doi: 10.1042/BST0360976.
5
A mitochondrial protein compendium elucidates complex I disease biology.
Cell. 2008 Jul 11;134(1):112-23. doi: 10.1016/j.cell.2008.06.016.
6
Generation of reactive oxygen species by mitochondrial complex I: implications in neurodegeneration.
Neurochem Res. 2008 Dec;33(12):2487-501. doi: 10.1007/s11064-008-9747-0. Epub 2008 Jun 6.
7
Assembly of mitochondrial complex I and defects in disease.
Biochim Biophys Acta. 2009 Jan;1793(1):78-88. doi: 10.1016/j.bbamcr.2008.04.015. Epub 2008 May 4.
8
Granzyme A cleaves a mitochondrial complex I protein to initiate caspase-independent cell death.
Cell. 2008 May 16;133(4):681-92. doi: 10.1016/j.cell.2008.03.032.
9
GRIM-19 is essential for maintenance of mitochondrial membrane potential.
Mol Biol Cell. 2008 May;19(5):1893-902. doi: 10.1091/mbc.e07-07-0683. Epub 2008 Feb 20.
10
Protection by the NDI1 gene against neurodegeneration in a rotenone rat model of Parkinson's disease.
PLoS One. 2008 Jan 16;3(1):e1433. doi: 10.1371/journal.pone.0001433.
Zotero 插件