Neurology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Figures Only
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow CME: Take the course for this article:
 Volume 55, Number 11, December 12, 2000
Right arrow Correspondence:
Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when Correspondence are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Schulz, J. B.
Right arrow Articles by Bogdanov, M.B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Schulz, J. B.
Right arrow Articles by Bogdanov, M.B.
Neurology 2000;55:1719-1721
© 2000 American Academy of Neurology
Brief Communications

Oxidative stress in patients with Friedreich ataxia

J. B. Schulz, MD, T. Dehmer, MD, L. Schöls, MD, H. Mende, C. Hardt, MD, M. Vorgerd, MD, K. Bürk, MD, W. Matson, PhD, J. Dichgans, MD, M.F. Beal, MD and M.B. Bogdanov, PhD

From the Department of Neurology and Medical School (Drs. Schulz, Dehmer, Bürk, and Dichgans, and H. Mende), University of Tübingen, Germany; Department of Neurology (Dr. Schöls), St. Josef Hospital, Ruhr-University, Bochum, Germany; Department of Human Genetics (Dr. Hardt), University of Essen, Germany; Department of Neurology (Dr. Vorgerd), Bergmannsheil Hospital, Ruhr-University, Bochum, Germany; ESA Inc. (Drs. Matson and Bogdanov), Chelmsford, MA; and Departments of Neurology and Neuroscience (Drs. Beal and Bogdanov), Weill Medical College & Cornell University and the New York Presbytarian Hospital, New York, NY.

Address correspondence and reprint requests to Dr. Jörg B. Schulz, Department of Neurology, University of Tübingen, Hoppe-Seyler-Str. 3, D-72076 Tübingen, Germany; e-mail: joerg.b.schulz{at}uni-tuebingen.de

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




This article has been cited by other articles:


Home page
 J. Biol. Chem.Home page
O. Gakh, D. Y. Smith IV, and G. Isaya
Assembly of the Iron-binding Protein Frataxin in Saccharomyces cerevisiae Responds to Dynamic Changes in Mitochondrial Iron Influx and Stress Level
J. Biol. Chem., November 14, 2008; 283(46): 31500 - 31510.
[Abstract] [Full Text] [PDF]

Home page
 Arch NeurolHome page
M. Pandolfo
Friedreich Ataxia
Arch Neurol, October 1, 2008; 65(10): 1296 - 1303.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
S. Al-Mahdawi, R. M. Pinto, O. Ismail, D. Varshney, S. Lymperi, C. Sandi, D. Trabzuni, and M. Pook
The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues
Hum. Mol. Genet., March 1, 2008; 17(5): 735 - 746.
[Abstract] [Full Text] [PDF]

Home page
 Arch NeurolHome page
N. A. Di Prospero, C. J. Sumner, S. R. Penzak, B. Ravina, K. H. Fischbeck, and J. P. Taylor
Safety, Tolerability, and Pharmacokinetics of High-Dose Idebenone in Patients With Friedreich Ataxia
Arch Neurol, June 1, 2007; 64(6): 803 - 808.
[Abstract] [Full Text] [PDF]

Home page
 Arch NeurolHome page
P. Ribai, F. Pousset, M.-L. Tanguy, S. Rivaud-Pechoux, I. Le Ber, F. Gasparini, P. Charles, A.-S. Beraud, M. Schmitt, M. Koenig, et al.
Neurological, Cardiological, and Oculomotor Progression in 104 Patients With Friedreich Ataxia During Long-term Follow-up
Arch Neurol, April 1, 2007; 64(4): 558 - 564.
[Abstract] [Full Text] [PDF]

Home page
 FASEB J.Home page
J. V. Llorens, J. A. Navarro, M. J. Martinez-Sebastian, M. K. Baylies, S. Schneuwly, J. A. Botella, and M. D. Molto
Causative role of oxidative stress in a Drosophila model of Friedreich ataxia
FASEB J, February 1, 2007; 21(2): 333 - 344.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
I. Condo, N. Ventura, F. Malisan, B. Tomassini, and R. Testi
A Pool of Extramitochondrial Frataxin That Promotes Cell Survival
J. Biol. Chem., June 16, 2006; 281(24): 16750 - 16756.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
O. Gakh, S. Park, G. Liu, L. Macomber, J. A. Imlay, G. C. Ferreira, and G. Isaya
Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity
Hum. Mol. Genet., February 1, 2006; 15(3): 467 - 479.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
S. M. Hersch, S. Gevorkian, K. Marder, C. Moskowitz, A. Feigin, M. Cox, P. Como, C. Zimmerman, M. Lin, L. Zhang, et al.
Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG
Neurology, January 24, 2006; 66(2): 250 - 252.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
H. Seznec, D. Simon, C. Bouton, L. Reutenauer, A. Hertzog, P. Golik, V. Procaccio, M. Patel, J.-C. Drapier, M. Koenig, et al.
Friedreich ataxia: the oxidative stress paradox
Hum. Mol. Genet., February 15, 2005; 14(4): 463 - 474.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
H. Seznec, D. Simon, L. Monassier, P. Criqui-Filipe, A. Gansmuller, P. Rustin, M. Koenig, and H. Puccio
Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia
Hum. Mol. Genet., May 15, 2004; 13(10): 1017 - 1024.
[Abstract] [Full Text] [PDF]

Home page
 J. Neurosci.Home page
D. Simon, H. Seznec, A. Gansmuller, N. Carelle, P. Weber, D. Metzger, P. Rustin, M. Koenig, and H. Puccio
Friedreich Ataxia Mouse Models with Progressive Cerebellar and Sensory Ataxia Reveal Autophagic Neurodegeneration in Dorsal Root Ganglia
J. Neurosci., February 25, 2004; 24(8): 1987 - 1995.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
G. Karthikeyan, J. H. Santos, M. A. Graziewicz, W. C. Copeland, G. Isaya, B. V. Houten, and M. A. Resnick
Reduction in frataxin causes progressive accumulation of mitochondrial damage
Hum. Mol. Genet., December 15, 2003; 12(24): 3331 - 3342.
[Abstract] [Full Text] [PDF]

Home page
 J. Biol. Chem.Home page
A. Pastore, G. Tozzi, L. M. Gaeta, E. Bertini, V. Serafini, S. D. Cesare, V. Bonetto, F. Casoni, R. Carrozzo, G. Federici, et al.
Actin Glutathionylation Increases in Fibroblasts of Patients with Friedreich's Ataxia: A POTENTIAL ROLE IN THE PATHOGENESIS OF THE DISEASE
J. Biol. Chem., October 24, 2003; 278(43): 42588 - 42595.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
G. Tan, E. Napoli, F. Taroni, and G. Cortopassi
Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells
Hum. Mol. Genet., July 15, 2003; 12(14): 1699 - 1711.
[Abstract] [Full Text] [PDF]

Home page
 FASEB J.Home page
M. S. COOKE, M. D. EVANS, M. DIZDAROGLU, and J. LUNEC
Oxidative DNA damage: mechanisms, mutation, and disease
FASEB J, July 1, 2003; 17(10): 1195 - 1214.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
A. Filla and A. J. Moss
Idebenone for treatment of Friedreich's ataxia?
Neurology, May 27, 2003; 60(10): 1569 - 1570.
[Full Text] [PDF]

Home page
 Hum Mol GenetHome page
M. L. Jauslin, T. Wirth, T. Meier, and F. Schoumacher
A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy
Hum. Mol. Genet., November 15, 2002; 11(24): 3055 - 3063.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
G. Karthikeyan, L. K. Lewis, and M. A. Resnick
The mitochondrial protein frataxin prevents nuclear damage
Hum. Mol. Genet., May 16, 2002; 11(11): 1351 - 1362.
[Abstract] [Full Text] [PDF]

Home page
 Arch NeurolHome page
D. R. Lynch, J. M. Farmer, L. J. Balcer, and R. B. Wilson
Friedreich Ataxia: Effects of Genetic Understanding on Clinical Evaluation and Therapy
Arch Neurol, May 1, 2002; 59(5): 743 - 747.
[Abstract] [Full Text] [PDF]

Home page
 Arch. Dis. Child.Home page
G Tozzi, M Nuccetelli, M Lo Bello, S Bernardini, L Bellincampi, S Ballerini, L M Gaeta, C Casali, A Pastore, G Federici, et al.
Antioxidant enzymes in blood of patients with Friedreich's ataxia
Arch. Dis. Child., May 1, 2002; 86(5): 376 - 379.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
S. A. Shoichet, A. T. Baumer, D. Stamenkovic, H. Sauer, A. F. H. Pfeiffer, C. R. Kahn, D. Muller-Wieland, C. Richter, and M. Ristow
Frataxin promotes antioxidant defense in a thiol-dependent manner resulting in diminished malignant transformation in vitro
Hum. Mol. Genet., April 1, 2002; 11(7): 815 - 821.
[Abstract] [Full Text] [PDF]

Home page
 HeartHome page
A O Hausse, Y Aggoun, D Bonnet, D Sidi, A Munnich, A Rotig, and P Rustin
Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia
Heart, April 1, 2002; 87(4): 346 - 349.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
P. Cavadini, H. A. O'Neill, O. Benada, and G. Isaya
Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia
Hum. Mol. Genet., February 1, 2002; 11(3): 217 - 227.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
K. Chantrel-Groussard, V. Geromel, H. Puccio, M. Koenig, A. Munnich, A. Rotig, and P. Rustin
Disabled early recruitment of antioxidant defenses in Friedreich's ataxia
Hum. Mol. Genet., September 1, 2001; 10(19): 2061 - 2067.
[Abstract] [Full Text] [PDF]

Home page
 NeurologyHome page
T. Sherer and J. T. Greenamyre
A therapeutic target and biomarker in Friedreich's ataxia
Neurology, December 12, 2000; 55(11): 1600 - 1601.
[Full Text] [PDF]


HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 2000 by AAN Enterprises, Inc.