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Friedreich's ataxia: Coenzyme Q(10) and vitamin E therapy

Cooper, JM; Schapira, AHV; (2007) Friedreich's ataxia: Coenzyme Q(10) and vitamin E therapy. MITOCHONDRION , 7 S127 - S135. 10.1016/j.mito.2007.04.001.

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Abstract

Since the identification of the genetic mutation causing Friedreich's ataxia (FRDA) our understanding of the mechanisms underlying disease pathogenesis have improved markedly. The genetic abnormality results in the deficiency of frataxin, a protein targeted to the mitochondrion. There is extensive evidence that mitochondrial respiratory chain dysfunction, oxidative damage and iron accumulation play significant roles in the disease mechanism. There remains considerable debate as to the normal function of frataxin, but it is likely to be involved in mitochondrial iron handling, antioxidant regulation, and/or iron sulphur centre regulation. Therapeutic avenues for patients with FRDA are beginning to be explored in particular targeting antioxidant protection, enhancement of mitochondrial oxidative phosphorylation, iron chelation and more recently increasing FRDA transcription. The use of quinone therapy has been the most extensively studied to date with clear benefits demonstrated using evaluations of both disease biomarkers and clinical symptoms, and this is the topic that will be covered in this review. (c) 2007 Elsevier B.V. on behalf of Mitochondria Research Society.

Type: Article
Title: Friedreich's ataxia: Coenzyme Q(10) and vitamin E therapy
DOI: 10.1016/j.mito.2007.04.001
Keywords: friedreich's ataxia, ataxia, coenzyme Q(10), mitochondria, neurodegeneration, TRIPLET-REPEAT EXPANSION, MAGNETIC-RESONANCE SPECTROSCOPY, MITOCHONDRIAL IRON HOMEOSTASIS, ELECTRON-TRANSPORT CHAIN, E-DEFICIENCY, OXIDATIVE STRESS, CONTROLLED-TRIAL, YEAST FRATAXIN, IDEBENONE TREATMENT, CLINICAL-FEATURES
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
URI: http://discovery.ucl.ac.uk/id/eprint/192515
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