Mitochondrial Diseases Part III: Therapeutic interventions in mouse models of OXPHOS deficiencies

Susana Peralta, Alessandra Torraco, Luisa Iommarini, Francisca Diaz

Research output: Contribution to journalArticlepeer-review


Mitochondrial defects are the cause of numerous disorders affecting the oxidative phosphorylation system (OXPHOS) in humans leading predominantly to neurological and muscular degeneration. The molecular origin, manifestations, and progression of mitochondrial diseases have a broad spectrum, which makes very challenging to find a globally effective therapy. The study of the molecular mechanisms underlying the mitochondrial dysfunction indicates that there is a wide range of pathways, enzymes and molecules that can be potentially targeted for therapeutic purposes. Therefore, focusing on the pathology of the disease is essential to design new treatments. In this review, we will summarize and discuss the different therapeutic interventions tested in some mouse models of mitochondrial diseases emphasizing the molecular mechanisms of action and their potential applications.

Original languageEnglish
Pages (from-to)71-80
Number of pages10
Publication statusPublished - 2015


  • Mitochondria
  • Mitochondrial diseases
  • Mouse models
  • Oxidative phosphorylation

ASJC Scopus subject areas

  • Cell Biology
  • Molecular Biology
  • Molecular Medicine


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