Protecting the mitochondrial powerhouse

Morten Scheibye-Knudsen, Evandro F. Fang, Deborah L. Croteau, David M. Wilson, Vilhelm A. Bohr*

*Corresponding author for this work
156 Citations (Scopus)

Abstract

Mitochondria are the oxygen-consuming power plants of cells. They provide a critical milieu for the synthesis of many essential molecules and allow for highly efficient energy production through oxidative phosphorylation. The use of oxygen is, however, a double-edged sword that on the one hand supplies ATP for cellular survival, and on the other leads to the formation of damaging reactive oxygen species (ROS). Different quality control pathways maintain mitochondria function including mitochondrial DNA (mtDNA) replication and repair, fusion-fission dynamics, free radical scavenging, and mitophagy. Further, failure of these pathways may lead to human disease. We review these pathways and propose a strategy towards a treatment for these often untreatable disorders.

Original languageEnglish
JournalTrends in Cell Biology
Volume25
Issue number3
Pages (from-to)158-170
Number of pages13
ISSN0962-8924
DOIs
Publication statusPublished - 1 Mar 2015
Externally publishedYes

Keywords

  • Disease
  • DNA repair
  • Mitochondria
  • Mitophagy
  • Reactive oxygen species

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