期刊
CELLULAR AND MOLECULAR LIFE SCIENCES
卷 77, 期 4, 页码 607-618出版社
SPRINGER BASEL AG
DOI: 10.1007/s00018-019-03293-0
关键词
Mitochondria; NADH; ubiquinone oxidoreductase; Complex I assembly; OXPHOS; Supercomplex; Drosophila
资金
- National Institute of Health [GM124717] Funding Source: Medline
- NIAMS NIH HHS [R21 AR077312] Funding Source: Medline
- NIDDK NIH HHS [R21 DK112074] Funding Source: Medline
- NIGMS NIH HHS [R35 GM124717] Funding Source: Medline
NADH:ubiquinone oxidoreductase, more commonly referred to as mitochondrial complex I (CI), is the largest discrete enzyme of the oxidative phosphorylation system (OXPHOS). It is localized to the mitochondrial inner membrane. CI oxidizes NADH generated from the tricarboxylic acid cycle to NAD(+), in a series of redox reactions that culminates in the reduction of ubiquinone, and the transport of protons from the matrix across the inner membrane to the intermembrane space. The resulting proton-motive force is consumed by ATP synthase to generate ATP, or harnessed to transport ions, metabolites and proteins into the mitochondrion. CI is also a major source of reactive oxygen species. Accordingly, impaired CI function has been associated with a host of chronic metabolic and degenerative disorders such as diabetes, cardiomyopathy, Parkinson's disease (PD) and Leigh syndrome. Studies on Drosophila have contributed to our understanding of the multiple roles of CI in bioenergetics and organismal physiology. Here, we explore and discuss some of the studies on Drosophila that have informed our understanding of this complex and conclude with some of the open questions about CI that can be resolved by studies on Drosophila.
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