The mitochondrial production of reactive oxygen species: mechanisms and implications in human pathology

IUBMB Life. Sep-Nov 2001;52(3-5):159-64. doi: 10.1080/15216540152845957.


Mitochondria are major sources of reactive oxygen species (ROS); the main sites of superoxide radical production in the respiratory chain are Complexes III and I; however, other mitochondrial enzymes, such as Complex II, glycerol-1-phosphate dehydrogenase, and dihydroorotate dehydrogenase, are also involved in production of ROS. ROS appear to be released both in the matrix and in the intermembrane space; however, their appearance outside the mitochondria may not be physiologically relevant. ROS production is increased in State 4 and in all conditions when the respiratory components are substantially in the reduced form. Accordingly, defects inducing decrease of electron transfer in the respiratory chain, as in many pathological conditions, are bound to enhance ROS production.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Electron Transport Complex I
  • Electron Transport Complex II
  • Electron Transport Complex III / metabolism
  • Glycerolphosphate Dehydrogenase / metabolism
  • Humans
  • Mitochondria / enzymology
  • Mitochondria / metabolism*
  • Multienzyme Complexes / metabolism
  • NADH, NADPH Oxidoreductases / metabolism
  • Oxidoreductases / metabolism
  • Oxidoreductases Acting on CH-CH Group Donors*
  • Reactive Oxygen Species / adverse effects*
  • Reactive Oxygen Species / metabolism*
  • Succinate Dehydrogenase / metabolism
  • Superoxides / metabolism


  • Multienzyme Complexes
  • Reactive Oxygen Species
  • Superoxides
  • Oxidoreductases
  • Glycerolphosphate Dehydrogenase
  • glycerol-1-phosphate dehydrogenase
  • Oxidoreductases Acting on CH-CH Group Donors
  • Electron Transport Complex II
  • dihydroorotate dehydrogenase
  • Succinate Dehydrogenase
  • NADH, NADPH Oxidoreductases
  • Electron Transport Complex I
  • Electron Transport Complex III