Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization
AbstractRespiratory 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
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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.
Sun D, Li B, Qiu R, Fang H, Lyu J. Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization. International Journal of Molecular Sciences. 2016; 17(6):926.Chicago/Turabian Style
Sun, Dayan; Li, Bin; Qiu, Ruyi; Fang, Hezhi; Lyu, Jianxin. 2016. "Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization." Int. J. Mol. Sci. 17, no. 6: 926.
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