Oxidative stress biomarkers in mitochondrial myopathies, basally and after cysteine donor supplementation

Michelangelo Mancuso, Daniele Orsucci, Annalisa LoGerfo, Anna Rocchi, Lucia Petrozzi, Claudia Nesti, Fabio Galetta, Gino Santoro, Luigi Murri, Gabriele Siciliano

Research output: Contribution to journalArticle

Abstract

Mitochondrial diseases are due to impairment of the mitochondrial respiratory chain. A plausible pathogenic mechanism leading to cellular dysfunction and phenotypic expression is oxidative stress, but there are surprisingly few clinical studies on this subject. Glutathione (GSH) deficiency has been reported in mitochondrial diseases, and the biosynthesis of glutathione depends on cysteine availability. We have examined oxidative stress biomarkers [advanced oxidation protein products (AOPP) and ferric reducing antioxidant power (FRAP)] in blood samples from 27 patients and 42 controls. AOPP levels were greater in patients than in controls (P value

Original languageEnglish
Pages (from-to)774-781
Number of pages8
JournalJournal of Neurology
Volume257
Issue number5
DOIs
Publication statusPublished - May 2010

Keywords

  • Mitochondrial disorders
  • Mitochondrial myopathy
  • mtDNA
  • Reactive oxygen species
  • ROS

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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