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Int. J. Mol. Sci. 2016, 17(6), 926; doi:10.3390/ijms17060926

Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization

Key Laboratory of Laboratory Medicine, Ministry of Education, Zhejiang Provincial Key Laboratory of Medical Genetics, College of Laboratory Medicine and Life Sciences, Wenzhou Medical University, Wenzhou 325035, China
These authors contributed equally to this work.
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Academic Editor: Anthony Lemarié
Received: 13 April 2016 / Revised: 31 May 2016 / Accepted: 6 June 2016 / Published: 21 June 2016
(This article belongs to the Section Biochemistry, Molecular and Cellular Biology)
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Abstract

Respiratory chain complexes are organized into large supercomplexes among which supercomplex In + IIIn + IVn is the only one that can directly transfer electrons from NADH to oxygen. Recently, it was reported that the formation of supercomplex In + IIIn + IVn in mice largely depends on their genetic background. However, in this study, we showed that the composition of supercomplex In + IIIn + IVn is well conserved in various mouse and human cell lines. Strikingly, we found that a minimal supercomplex In + IIIn, termed “lowest supercomplex” (LSC) in this study because of its migration at the lowest position close to complex V dimers in blue native polyacrylamide gel electrophoresis, was associated with complex IV to form a supercomplex In + IIIn + IVn in some, but not all of the human and mouse cells. In addition, we observed that the 3697G>A mutation in mitochondrial-encoded NADH dehydrogenase 1 (ND1) in one patient with Leigh’s disease specifically affected the assembly of supercomplex In + IIIn + IVn containing LSC, leading to decreased cellular respiration and ATP generation. In conclusion, we showed the existence of LSC In + IIIn + IVn and impairment of this supercomplex causes disease. View Full-Text
Keywords: mitochondrial supercomplex; nuclear genetic background; respiratory chain; mitochondrial dysfunction; Leigh’s disease mitochondrial supercomplex; nuclear genetic background; respiratory chain; mitochondrial dysfunction; Leigh’s disease
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Sun, D.; Li, B.; Qiu, R.; Fang, H.; Lyu, J. Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization. Int. J. Mol. Sci. 2016, 17, 926.

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