Cited by 830Mitochondrial Function and PathologyCoenzyme Q10 studies and effectsATP Synthase and ATPases Research

Supercomplex assembly determines electron flux in the mitochondrial electron transport chain

Open access
Lapuente-Brun, Esther, Moreno-Loshuertos, Raquel, Acín-Pérez, Rebeca, Latorre-Pellicer, Ana, Colás, Carmen, Balsa, Eduardo, Perales-Clemente, Ester, Quirós, Pedro M. +10 more·2013·Science

Abstract

The textbook description of mitochondrial respiratory complexes (RCs) views them as free-moving entities linked by the mobile carriers coenzyme Q (CoQ) and cytochrome c (cyt c). This model (known as the fluid model) is challenged by the proposal that all RCs except complex II can associate in supercomplexes (SCs). The proposed SCs are the respirasome (complexes I, III, and IV), complexes I and III, and complexes III and IV. The role of SCs is unclear, and their existence is debated. By genetic modulation of interactions between complexes I and III and III and IV, we show that these associations define dedicated CoQ and cyt c pools and that SC assembly is dynamic and organizes electron flux to optimize the use of available substrates.

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Crossref · 10.1126/science.1230381

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Authors
Lapuente-Brun, E., Moreno-Loshuertos, R., Acín-Pérez, R., Latorre-Pellicer, A., Colás, C., Balsa, E., Perales-Clemente, E., Quirós, P. M., Calvo, E., Rodríguez-Hernández, M. A., Navas, P., Cruz, R., Carracedo, Á., López-Otín, C., Pérez-Martos, A., Fernández-Silva, P., Fernández-Vizarra, E., & Enríquez, J. A.
Affiliations
  • Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid, Spain.
  • Departamento de Bioquímica y Biología Molecular y Celular, Facultad de Ciencias, Universidad de Zaragoza, Zaragoza 50009, Spain.
  • Spanish National Centre for Cardiovascular Research (ES)
  • Universidad de Oviedo (ES)
  • Universidad de Zaragoza (ES)
  • Centro Andaluz de Biología del Desarrollo, Universidad Pablo de Olavide-CSIC and Centro de Investigación Biomédica en Red de Enfermedades Raras, ISCIII, Sevilla 41013 Spain.
  • Instituto de Salud Carlos III (ES)
  • Universidade de Santiago de Compostela (ES)
  • Departamento de Bioquímica y Biología Molecular, Instituto Universitario de Oncología (IUOPA), Universidad de Oviedo, 33006-Oviedo, Spain.
  • IIS Aragón, Unidad de Investigación Traslacional Instituto Aragones de Ciencias de la Salud, Hospital Universitario Miguel Servet, Zaragoza 50009, Spain.
Venue
Science
Published
2013-06-28
Volume
340
Issue
6140
Pages
1567-1570
ISSN
  • 0036-8075 (ISSN-L)
  • 1095-9203
OpenAlex
W1965285497
Cited by
830
References
27
Citations by year
2026: 49 · 2025: 41 · 2024: 55 · 2023: 72 · 2022: 50 · 2021: 84 · 2020: 83 · 2019: 57 · 2018: 62 · 2017: 58 · 2016: 82 · 2015: 61 · 2014: 57 · 2013: 19
FWCI
32.67
Type
journal article
Language
en
Open access
Licence
cc-by-nc-nd
Locations
  • Science (publishedVersion)
  • PubMed (publishedVersion)
  • Zaguan (University of Zaragoza Repository) (publishedVersion)
  • Consultation of the Doctoral Thesis Database (TESEO) (Ministerio de Educación, Cultura y Deporte) (submittedVersion, cc-by-nc-nd)
  • DIGITAL.CSIC (Spanish National Research Council (CSIC)) (submittedVersion)
  • RUNA. Repositorio Institucional del Sergas (Servicio Gallego de Salud (SERGAS)) (submittedVersion, other-oa)
  • Padua Research Archive (University of Padova) (submittedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › ATP Synthase and ATPases Research
Keywords
mitochondrial supercomplexes, respiratory chain complexes, electron transport chain, coenzyme Q10, cytochrome c, respirasome, complex I, cytochrome bc1 complex, cytochrome c oxidase, electron flux, complex assembly
MeSH terms
Amino Acid Sequence, Animals, Cells, Cultured, Electron Transport Complex IV, Electron Transport, Humans, Mice, Inbred C57BL, Mitochondria, Molecular Sequence Data, Electron Transport Complex III, Ubiquinone, Electron Transport Complex I, Cytochromes c, Mice, Gene Knockdown Techniques, HEK293 Cells

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