Does oxidative stress contribute to the pathology of Friedreich's ataxia? A radical question

FASEB J. 2010 Jul;24(7):2152-63. doi: 10.1096/fj.09-143222. Epub 2010 Mar 10.

Abstract

Friedreich's ataxia (FRDA) is a hereditary neurodegenerative disease that frequently culminates in cardiac failure at an early age. FRDA is believed to arise from reduced synthesis of the mitochondrial iron chaperone frataxin due to impaired gene transcription, which leads to mitochondrial iron accumulation, dysfunction of mitochondrial Fe-S containing enzymes, and increased Fenton-mediated free radical production. Recent reports have challenged this generally accepted hypothesis, by suggesting that the oxidative stress component in FRDA is minimal and thereby questioning the benefit of antioxidant therapeutic strategies. We suggest that this apparent paradox results from the radically divergent chemistries of the participating reactive oxygen species (ROS), the major cellular subcompartments involved and the overall cellular responses to ROS. In this review, we consider these factors and conclude that oxidative stress does constitute a major contributing factor to FRDA pathology. This reaffirms the idea that the rational design of specific small molecule multifunctional antioxidants will benefit FRDA patients.

Publication types

  • Review

MeSH terms

  • Energy Metabolism
  • Frataxin
  • Friedreich Ataxia / metabolism*
  • Friedreich Ataxia / pathology
  • Humans
  • Iron-Binding Proteins / physiology
  • Iron-Sulfur Proteins / physiology
  • Oxidation-Reduction
  • Oxidative Stress*

Substances

  • Iron-Binding Proteins
  • Iron-Sulfur Proteins