Mitochondrial cytochrome c oxidase biogenesis: Recent developments

Alba Timón-Gómez, Eva Nývltová, Luciano A. Abriata, Alejandro J. Vila, Jonathan Hosler, Antoni Barrientos

Research output: Contribution to journalReview articlepeer-review

131 Scopus citations


Mitochondrial cytochrome c oxidase (COX) is the primary site of cellular oxygen consumption and is essential for aerobic energy generation in the form of ATP. Human COX is a copper-heme A hetero-multimeric complex formed by 3 catalytic core subunits encoded in the mitochondrial DNA and 11 subunits encoded in the nuclear genome. Investigations over the last 50 years have progressively shed light into the sophistication surrounding COX biogenesis and the regulation of this process, disclosing multiple assembly factors, several redox-regulated processes leading to metal co-factor insertion, regulatory mechanisms to couple synthesis of COX subunits to COX assembly, and the incorporation of COX into respiratory supercomplexes. Here, we will critically summarize recent progress and controversies in several key aspects of COX biogenesis: linear versus modular assembly, the coupling of mitochondrial translation to COX assembly and COX assembly into respiratory supercomplexes.

Original languageEnglish (US)
Pages (from-to)163-178
Number of pages16
JournalSeminars in Cell and Developmental Biology
StatePublished - Apr 2018


  • COX assembly factor
  • COX1
  • COX2
  • Mitochondrial cytochrome c oxidase
  • Respiratory supercomplex

ASJC Scopus subject areas

  • Developmental Biology
  • Cell Biology


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