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Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization
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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4926459/ https://www.ncbi.nlm.nih.gov/pubmed/27338358 http://dx.doi.org/10.3390/ijms17060926 |
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author | Sun, Dayan Li, Bin Qiu, Ruyi Fang, Hezhi Lyu, Jianxin |
author_facet | Sun, Dayan Li, Bin Qiu, Ruyi Fang, Hezhi Lyu, Jianxin |
author_sort | Sun, Dayan |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-4926459 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-49264592016-07-06 Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization Sun, Dayan Li, Bin Qiu, Ruyi Fang, Hezhi Lyu, Jianxin Int J Mol Sci Article 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. MDPI 2016-06-21 /pmc/articles/PMC4926459/ /pubmed/27338358 http://dx.doi.org/10.3390/ijms17060926 Text en © 2016 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC-BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Sun, Dayan Li, Bin Qiu, Ruyi Fang, Hezhi Lyu, Jianxin Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization |
title | Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization |
title_full | Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization |
title_fullStr | Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization |
title_full_unstemmed | Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization |
title_short | Cell Type-Specific Modulation of Respiratory Chain Supercomplex Organization |
title_sort | cell type-specific modulation of respiratory chain supercomplex organization |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4926459/ https://www.ncbi.nlm.nih.gov/pubmed/27338358 http://dx.doi.org/10.3390/ijms17060926 |
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