Mitochondria in lung biology and pathology: more than just a powerhouse

Am J Physiol Lung Cell Mol Physiol. 2014 Jun 1;306(11):L962-74. doi: 10.1152/ajplung.00073.2014. Epub 2014 Apr 18.


An explosion of new information about mitochondria reveals that their importance extends well beyond their time-honored function as the "powerhouse of the cell." In this Perspectives article, we summarize new evidence showing that mitochondria are at the center of a reactive oxygen species (ROS)-dependent pathway governing the response to hypoxia and to mitochondrial quality control. The potential role of the mitochondrial genome as a sentinel molecule governing cytotoxic responses of lung cells to ROS stress also is highlighted. Additional attention is devoted to the fate of damaged mitochondrial DNA relative to its involvement as a damage-associated molecular pattern driving adverse lung and systemic cell responses in severe illness or trauma. Finally, emerging strategies for replenishing normal populations of mitochondria after damage, either through promotion of mitochondrial biogenesis or via mitochondrial transfer, are discussed.

Keywords: hypoxia; lung injury; mitochondrial biogenesis; mitochondrial transfer; mtDNA.

Publication types

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

MeSH terms

  • Animals
  • Biomarkers
  • DNA Damage
  • DNA, Mitochondrial / blood
  • DNA, Mitochondrial / genetics
  • Humans
  • Lung / metabolism
  • Lung / pathology*
  • Mitochondria / physiology*
  • Oxidative Stress
  • Oxygen / metabolism
  • Reactive Oxygen Species / metabolism
  • Respiratory Distress Syndrome / blood


  • Biomarkers
  • DNA, Mitochondrial
  • Reactive Oxygen Species
  • Oxygen