Suppression mechanisms of COX assembly defects in yeast and human: Insights into the COX assembly process

Antonio Barrientos, Karine Gouget, Darryl Horn, Ileana C. Soto, Flavia Fontanesi

Research output: Contribution to journalArticle

76 Citations (Scopus)

Abstract

Eukaryotic cytochrome c oxidase (COX) is the terminal enzyme of the mitochondrial respiratory chain. COX is a multimeric enzyme formed by subunits of dual genetic origin whose assembly is intricate and highly regulated. In addition to the structural subunits, a large number of accessory factors are required to build the holoenzyme. The function of these factors is required in all stages of the assembly process. They are relevant to human health because devastating human disorders have been associated with mutations in nuclear genes encoding conserved COX assembly factors. The study of yeast strains and human cell lines from patients carrying mutations in structural subunits and COX assembly factors has been invaluable to attain the current state of knowledge, even if still fragmentary, of the COX assembly process. After the identification of the genes involved, the isolation and characterization of genetic and metabolic suppressors of COX assembly defects, reviewed here, have become a profitable strategy to gain insight into their functions and the pathways in which they operate. Additionally, they have the potential to provide useful information for devising therapeutic approaches to combat human disorders associated with COX deficiency.

Original languageEnglish
Pages (from-to)97-107
Number of pages11
JournalBiochimica et Biophysica Acta - Molecular Cell Research
Volume1793
Issue number1
DOIs
StatePublished - Jan 1 2009

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Oxidoreductases
Yeasts
Combat Disorders
Holoenzymes
Mutation
Electron Transport Complex IV
Enzymes
Electron Transport
Genes
Cell Line
Health
Therapeutics

Keywords

  • COX assembly
  • Cytochrome c oxidase
  • Mitochondria
  • Mitochondrial disorder
  • Suppression

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology

Cite this

Suppression mechanisms of COX assembly defects in yeast and human : Insights into the COX assembly process. / Barrientos, Antonio; Gouget, Karine; Horn, Darryl; Soto, Ileana C.; Fontanesi, Flavia.

In: Biochimica et Biophysica Acta - Molecular Cell Research, Vol. 1793, No. 1, 01.01.2009, p. 97-107.

Research output: Contribution to journalArticle

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