Biogenesis of cytochrome oxidase-sophisticated assembly lines in the mitochondrial inner membrane

Gene. 2005 Jul 18;354:43-52. doi: 10.1016/j.gene.2005.03.017.


Biogenesis of the cytochrome oxidase complex in the mitochondrial inner membrane depends on the concerted action of a variety of proteins. Recent studies shed light on this biological assembly process revealing an astonishingly complex procedure by which the different subunits of the enzymes are put together and the required cofactors are supplied. In this review we present a hypothetical model for the assembly process of cytochrome oxidase based on the current knowledge of the functions of specific assembly factors. According to this model the two largest subunits of the complex are first equipped with their respective cofactors on independent assembly lines. Prior to their assembly with the residual subunits that complete the whole complex, these two subcomplexes remain bound to substrate-specific chaperones. We propose that these chaperones, Mss51 for subunit 1 and Cox20 for subunit 2, control the coordinate assembly process to prevent potentially harmful redox reactions of unassembled or misassembled subunits.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Amino Acid Sequence
  • Copper / metabolism
  • Electron Transport Complex IV / biosynthesis*
  • Electron Transport Complex IV / genetics
  • Electron Transport Complex IV / metabolism
  • Intracellular Membranes / enzymology
  • Intracellular Membranes / metabolism*
  • Membrane Proteins / metabolism
  • Mitochondria / enzymology
  • Mitochondria / genetics
  • Mitochondria / metabolism*
  • Mitochondrial Proteins / biosynthesis*
  • Mitochondrial Proteins / genetics
  • Mitochondrial Proteins / metabolism
  • Models, Biological
  • Molecular Sequence Data
  • Nuclear Proteins / metabolism
  • Saccharomyces cerevisiae / enzymology
  • Saccharomyces cerevisiae / genetics
  • Saccharomyces cerevisiae / metabolism
  • Saccharomyces cerevisiae Proteins / metabolism
  • Sequence Homology, Amino Acid


  • Membrane Proteins
  • Mitochondrial Proteins
  • Nuclear Proteins
  • OXA1 protein
  • SCO1 protein, S cerevisiae
  • SHY1 protein, S cerevisiae
  • Saccharomyces cerevisiae Proteins
  • Copper
  • Electron Transport Complex IV