Oxidative stress in patients with Friedreich ataxia
JB Schulz, T Dehmer, L Schols, H Mende, C Hardt… - Neurology, 2000 - AAN Enterprises
Neurology, 2000•AAN Enterprises
Article abstract Increased generation of reactive oxygen species may underlie the
pathophysiology of Friedreich ataxia (FRDA). The authors measured concentrations of 8-
hydroxy-2'-deoxyguanosine (8OH2'dG), a marker of oxidative DNA damage, in urine and of
dihydroxybenzoic acid (DHBA), a marker of hydroxyl radical attack, in plasma of 33 patients
with FRDA. They found a 2.6-fold increase in normalized urinary 8OH2'dG but no change in
plasma DHBA as compared with controls. Oral treatment with 5 mg/kg/day of the antioxidant …
pathophysiology of Friedreich ataxia (FRDA). The authors measured concentrations of 8-
hydroxy-2'-deoxyguanosine (8OH2'dG), a marker of oxidative DNA damage, in urine and of
dihydroxybenzoic acid (DHBA), a marker of hydroxyl radical attack, in plasma of 33 patients
with FRDA. They found a 2.6-fold increase in normalized urinary 8OH2'dG but no change in
plasma DHBA as compared with controls. Oral treatment with 5 mg/kg/day of the antioxidant …
Article abstract Increased generation of reactive oxygen species may underlie the pathophysiology of Friedreich ataxia (FRDA). The authors measured concentrations of 8-hydroxy-2’-deoxyguanosine (8OH2’dG), a marker of oxidative DNA damage, in urine and of dihydroxybenzoic acid (DHBA), a marker of hydroxyl radical attack, in plasma of 33 patients with FRDA. They found a 2.6-fold increase in normalized urinary 8OH2’dG but no change in plasma DHBA as compared with controls. Oral treatment with 5 mg/kg/day of the antioxidant idebenone for 8 weeks significantly decreased urinary 8OH2’dG concentrations, indicating that 8OH2’dG may be useful in monitoring therapeutic interventions in patients with FRDA.–1721
American Academy of Neurology