Does oxidative stress contribute to the pathology of Friedreich’s ataxia? A radical question

Reduction of Hydrophilic Ubiquinones by the Flavin in Mitochondrial NADH:Ubiquinone Oxidoreductase (Complex I) and Production of Reactive Oxygen Species†

(2009) Martin S. King et al.   BIOCHEMISTRY

Copy number variants, diseases and gene expression

(2009) C. N. Henrichsen et al.   HUMAN MOLECULAR GENETICS

Multicellular models of Friedreich ataxia

(2009) Hélène Puccio   JOURNAL OF NEUROLOGY

The pathogenesis of Friedreich ataxia and the structure and function of frataxin

(2009) Massimo Pandolfo et al.   JOURNAL OF NEUROLOGY

Bacterial frataxin CyaY is the gatekeeper of iron-sulfur cluster formation catalyzed by IscS

(2009) Salvatore Adinolfi et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Impaired Nuclear Nrf2 Translocation Undermines the Oxidative Stress Response in Friedreich Ataxia

(2009) Vincent Paupe et al.   PLoS One

Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant

(2009) M. L.-H. Huang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

NRF2 and KEAP1 mutations: permanent activation of an adaptive response in cancer

(2009) John D. Hayes et al.   TRENDS IN BIOCHEMICAL SCIENCES

Production of Superoxide Anions by Keratinocytes Initiates P. acnes-Induced Inflammation of the Skin

(2009) Philippe A. Grange et al.   PLoS Pathogens

Iron–sulfur cluster biosynthesis in bacteria: Mechanisms of cluster assembly and transfer

(2008) Marc Fontecave et al.   ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation

(2008) O. Kakhlon et al.   BLOOD

Lipid peroxidation of membrane phospholipids generates hydroxy-alkenals and oxidized phospholipids active in physiological and/or pathological conditions

(2008) Angel Catalá   CHEMISTRY AND PHYSICS OF LIPIDS

Coenzyme Q10and vitamin E deficiency in Friedreich’s ataxia: predictor of efficacy of vitamin E and coenzyme Q10therapy

(2008) J. M. Cooper et al.   EUROPEAN JOURNAL OF NEUROLOGY

Glutathione-dependent redox status of frataxin-deficient cells in a yeast model of Friedreich's ataxia

(2008) F. Auchere et al.   HUMAN MOLECULAR GENETICS

Binding of Yeast Frataxin to the Scaffold for Fe-S Cluster Biogenesis, Isu

(2008) Tao Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Quality of potent Mn porphyrin-based SOD mimics and peroxynitrite scavengers for pre-clinical mechanistic/therapeutic purposes

(2008) Júlio S. Rebouças et al.   JOURNAL OF PHARMACEUTICAL AND BIOMEDICAL ANALYSIS

HDAC Inhibitors Correct Frataxin Deficiency in a Friedreich Ataxia Mouse Model

(2008) Myriam Rai et al.   PLoS One

The MCK mouse heart model of Friedreich's ataxia: Alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation

(2008) M. Whitnall et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia

(2008) P. R. Anderson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin

(2007) Isabella Zanella et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE