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Mitochondrial Respiratory Complex I
Li Fengjie1, Shen Lijun1, Fang Hezhi1*, Bai Yidong1,2*
1School of Laboratory Medicine and Life Science, Wenzhou Medical University, Wenzhou 325035, China; 2University of Texas Health Science Center, San Antonio 78229, USA
Abstract: Mitochondrial respiratory complex I (NADH: ubiquinone oxidoreductase) is the initial and rate limiting enzyme in electron transfer chain (ETC). In all five respiratory complexes， complex I is the largest enzyme with a molecular weight about 1 MDa. Mutations in both 45 subunits of complex I and its assemble factors have been implicated in many diseases. To provide a comprehensive understanding of complex I in human diseases， the complex I structure and the assembly of complex I were discussed in this review.
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