Oxidative stress in patients with Friedreich ataxia.
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
Schulz
J B
JB
Department of Neurology, University of Tübingen, Germany. joerg.b.schulz@uni-tuebingen.de
Dehmer
T
T
Schöls
L
L
Mende
H
H
Hardt
C
C
Vorgerd
M
M
Bürk
K
K
Matson
W
W
Dichgans
J
J
Beal
M F
MF
Bogdanov
M B
MB
D000080242:8-Hydroxy-2'-Deoxyguanosine; D000328:Adult; D003849:Deoxyguanosine / Q000031:analogs & derivatives* / Q000652:urine*; D005621:Friedreich Ataxia / Q000235:genetics / Q000652:urine*; D006801:Humans; D018384:Oxidative Stress
D000080242:8-Hydroxy-2'-Deoxyguanosine; D003849:Deoxyguanosine