title: The Enigma of the Respiratory Chain Supercomplex.
abstract: Respiratory chain dysfunction plays an important role in human disease and aging. It is now well established that the individual respiratory complexes can be organized into supercomplexes, and structures for these macromolecular assemblies, determined by electron cryo-microscopy, have been described recently. Nevertheless, the reason why supercomplexes exist remains an enigma. The widely held view that they enhance catalysis by channeling substrates is challenged by both structural and biophysical information. Here, we evaluate and discuss data and hypotheses on the structures, roles, and assembly of respiratory-chain supercomplexes and propose a future research agenda to address unanswered questions.
authors.affiliation: Department of Mitochondrial Biology, Max Planck Institute for Biology of Ageing, Joseph-Stelzmann-Strasse 9b, 50931 Cologne, Germany.
authors.forename: Dusanka
authors.initials: D
authors.lastname: Milenkovic
authors.affiliation: Medical Research Council Mitochondrial Biology Unit, University of Cambridge, Wellcome Trust/MRC Building, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0XY, UK.
authors.forename: James N
authors.initials: JN
authors.lastname: Blaza
authors.affiliation: Department of Mitochondrial Biology, Max Planck Institute for Biology of Ageing, Joseph-Stelzmann-Strasse 9b, 50931 Cologne, Germany; Department of Medical Biochemistry and Biophysics, Karolinska Institutet, 171 77 Stockholm, Sweden. Electronic address: nils-goran.larsson@ki.se.
authors.forename: Nils-Göran
authors.initials: NG
authors.lastname: Larsson
authors.affiliation: Medical Research Council Mitochondrial Biology Unit, University of Cambridge, Wellcome Trust/MRC Building, Cambridge Biomedical Campus, Hills Road, Cambridge, CB2 0XY, UK. Electronic address: jh@mrc-mbu.cam.ac.uk.
authors.forename: Judy
authors.initials: J
authors.lastname: Hirst
mesh_terms: D000818:Animals; D004579:Electron Transport; D045222:Electron Transport Chain Complex Proteins / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D008322:Mammals / Q000378:metabolism; D008954:Models, Biological; D008958:Models, Molecular; D055550:Protein Stability
chemical_list: D045222:Electron Transport Chain Complex Proteins