Identification of a protein mediating respiratory supercomplex stability Academic Article uri icon


MeSH Major

  • Cell Respiration
  • Multienzyme Complexes


  • The complexes of the electron transport chain associate into large macromolecular assemblies, which are believed to facilitate efficient electron flow. We have identified a conserved mitochondrial protein, named respiratory supercomplex factor 1 (Rcf1-Yml030w), that is required for the normal assembly of respiratory supercomplexes. We demonstrate that Rcf1 stably and independently associates with both Complex III and Complex IV of the electron transport chain. Deletion of the RCF1 gene caused impaired respiration, probably as a result of destabilization of respiratory supercomplexes. Consistent with the hypothetical function of these respiratory assemblies, loss of RCF1 caused elevated mitochondrial oxidative stress and damage. Finally, we show that knockdown of HIG2A, a mammalian homolog of RCF1, causes impaired supercomplex formation. We suggest that Rcf1 is a member of an evolutionarily conserved protein family that acts to promote respiratory supercomplex assembly and activity.

publication date

  • March 7, 2012



  • Academic Article



  • eng

PubMed Central ID

  • PMC3302151

Digital Object Identifier (DOI)

  • 10.1016/j.cmet.2012.02.006

PubMed ID

  • 22405070

Additional Document Info

start page

  • 348

end page

  • 60


  • 15


  • 3