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Cytochrome c oxidase deficiency.

Michele Brischigliaro1, Massimo Zeviani2

  • 1Department of Biology, University of Padova, Padova, Italy.

Biochimica Et Biophysica Acta. Bioenergetics
|November 10, 2020
PubMed
Summary
This summary is machine-generated.

Cytochrome c oxidase (COX) deficiency presents diverse genetic and clinical features due to enzyme complexity. Research highlights over 30 genes linked to COX deficiency, impacting various tissues and ages.

Keywords:
Cytochrome c oxidaseDisease modelsGeneticsMitochondrial diseasesOXPHOS

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Area of Science:

  • Biochemistry
  • Genetics
  • Molecular Biology

Background:

  • Cytochrome c oxidase (COX) deficiency exhibits significant genetic and phenotypic variability.
  • This heterogeneity stems from the enzyme's complex structure, post-translational modifications, and numerous assembly factors.

Purpose of the Study:

  • To review current knowledge on COX in health and disease.
  • To focus on the genetic causes and clinical manifestations of COX deficiency.
  • To explore insights from in vivo models.

Main Methods:

  • Literature review of genetic aetiology and clinical manifestations.
  • Analysis of biochemical signatures and functional features.
  • Examination of data from experimental in vivo models (yeast, fly, mouse, fish).

Main Results:

  • Over 30 genes are currently associated with COX deficiency, with ongoing discoveries.
  • COX deficiency presents with variable severity, affecting specific tissues or the whole organism.
  • Disease onset ranges from birth to late adulthood.

Conclusions:

  • Understanding COX deficiency requires integrating genetic, biochemical, and clinical data.
  • In vivo models are crucial for elucidating functional consequences and phenotypical outcomes.
  • Further research is needed to complete the gene list and refine understanding of COX-related disorders.