Protecting the mitochondrial powerhouse

Trends Cell Biol. 2015 Mar;25(3):158-70. doi: 10.1016/j.tcb.2014.11.002. Epub 2014 Dec 11.


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.

Keywords: DNA repair; disease; mitochondria; mitophagy; reactive oxygen species.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Animals
  • DNA Repair
  • DNA Replication
  • DNA, Mitochondrial / genetics
  • Homeostasis
  • Humans
  • Mitochondria / physiology*
  • Mitophagy
  • Oxidative Stress
  • Reactive Oxygen Species / metabolism


  • DNA, Mitochondrial
  • Reactive Oxygen Species