Evaluation of systemic redox states in patients carrying the MELAS A3243G mutation in mitochondrial DNA

Eur Neurol. 2012;67(4):232-7. doi: 10.1159/000336568. Epub 2012 Mar 14.

Abstract

Background/aims: To clarify the change of systemic redox states in patients carrying the A3243G mutation in mitochondrial DNA (A3243G), we evaluated oxidative stress and antioxidant activity in the serum of patients.

Methods: Oxidative stress and antioxidant activity in the serum samples obtained from 14 patients carrying A3243G and from 34 healthy controls were analyzed using the diacron-reactive oxygen metabolites (d-ROMs) and biological antioxidant potential (BAP) tests, respectively.

Results: The mean d-ROMs level of all patients was significantly greater than that of the controls (p < 0.005), and the mean BAP/d-ROMs ratio of all patients was significantly lower than that of the controls (p < 0.02). In the patients with a history of stroke-like episodes (n = 10), both mean d-ROMs and BAP levels were increased compared with those of the controls (both p < 0.01). The mean BAP level of the patients without a history of stroke-like episodes (n = 4) was significantly decreased compared with that of the controls (p < 0.001), but the mean d-ROMs levels were not significantly different.

Conclusion: d-ROMs and BAP tests indicated that patients carrying A3243G are always exposed to underlying oxidative stress, even at a remission state of stroke-like episodes.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Alanine / genetics
  • Antioxidants / metabolism
  • DNA, Mitochondrial / genetics*
  • Female
  • Glycine / genetics
  • Humans
  • Hydrogen Peroxide / blood
  • MELAS Syndrome / blood
  • MELAS Syndrome / genetics*
  • MELAS Syndrome / physiopathology*
  • Male
  • Mutation / genetics*
  • Oxidation-Reduction
  • Oxidative Stress / genetics*
  • Reactive Oxygen Species / blood
  • Statistics, Nonparametric
  • Young Adult

Substances

  • Antioxidants
  • DNA, Mitochondrial
  • Reactive Oxygen Species
  • Hydrogen Peroxide
  • Alanine
  • Glycine