Assembly of the oxidative phosphorylation system in humans: What we have learned by studying its defects

Erika Fernández-Vizarra, Valeria Tiranti, Massimo Zeviani

Research output: Contribution to journalArticle


Assembly of the oxidative phosphorylation (OXPHOS) system in the mitochondrial inner membrane is an intricate process in which many factors must interact. The OXPHOS system is composed of four respiratory chain complexes, which are responsible for electron transport and generation of the proton gradient in the mitochondrial intermembrane space, and of the ATP synthase that uses this proton gradient to produce ATP. Mitochondrial human disorders are caused by dysfunction of the OXPHOS system, and many of them are associated with altered assembly of one or more components of the OXPHOS system. The study of assembly defects in patients has been useful in unraveling and/or gaining a complete understanding of the processes by which these large multimeric complexes are formed. We review here current knowledge of the biogenesis of OXPHOS complexes based on investigation of the corresponding disorders.

Original languageEnglish
Pages (from-to)200-211
Number of pages12
JournalBiochimica et Biophysica Acta - Molecular Cell Research
Issue number1
Publication statusPublished - Jan 2009



  • Assembly
  • Electron transfer chain
  • Mitochondrial disorder
  • Mitochondrial DNA
  • Mitochondrial inner membrane
  • Mitochondrial respiratory chain
  • Oxidative phosphorylation
  • Respiratory chain complex

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology

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