Supercomplex assembly determines electron flux in the mitochondrial electron transport chain

Science. 2013 Jun 28;340(6140):1567-70. doi: 10.1126/science.1230381.

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.

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Cells, Cultured
  • Cytochromes c / metabolism*
  • Electron Transport
  • Electron Transport Complex I / genetics
  • Electron Transport Complex I / metabolism*
  • Electron Transport Complex III / genetics
  • Electron Transport Complex III / metabolism*
  • Electron Transport Complex IV / genetics
  • Electron Transport Complex IV / metabolism*
  • Gene Knockdown Techniques
  • HEK293 Cells
  • Humans
  • Mice
  • Mice, Inbred C57BL
  • Mitochondria / enzymology*
  • Molecular Sequence Data
  • Ubiquinone / metabolism*

Substances

  • Ubiquinone
  • Cytochromes c
  • Cox7a2 protein, mouse
  • Electron Transport Complex IV
  • Electron Transport Complex I
  • Electron Transport Complex III