Literature DB >> 17988688

Antioxidant use in Friedreich ataxia.

Lauren Myers1, Jennifer M Farmer, Robert B Wilson, Lisa Friedman, Amy Tsou, Susan L Perlman, Sub H Subramony, Christopher M Gomez, Tetsuo Ashizawa, George R Wilmot, Katherine D Mathews, Laura J Balcer, David R Lynch.   

Abstract

Many antioxidants have been suggested as potential treatments for Friedreich ataxia, but have not been tested in clinical trials. We found that a majority of patients in our cohort already use such antioxidants, including idebenone, which is not available at a pharmaceutical grade in the United States. Younger age, cardiomyopathy and shorter GAA repeat length were independent predictors of idebenone use, but no factors predicted use of other antioxidants. This confirms that non-prescription antioxidant use represents a major confounder to formal trials of existing and novel agents for Friedreich ataxia.

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Year:  2007        PMID: 17988688      PMCID: PMC2279016          DOI: 10.1016/j.jns.2007.10.008

Source DB:  PubMed          Journal:  J Neurol Sci        ISSN: 0022-510X            Impact factor:   3.181


  15 in total

Review 1.  Friedreich ataxia: effects of genetic understanding on clinical evaluation and therapy.

Authors:  David R Lynch; Jennifer M Farmer; Laura J Balcer; Robert B Wilson
Journal:  Arch Neurol       Date:  2002-05

2.  Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia.

Authors:  R Lodi; P E Hart; B Rajagopalan; D J Taylor; J G Crilley; J L Bradley; A M Blamire; D Manners; P Styles; A H Schapira; J M Cooper
Journal:  Ann Neurol       Date:  2001-05       Impact factor: 10.422

3.  Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia.

Authors:  A Rötig; P de Lonlay; D Chretien; F Foury; M Koenig; D Sidi; A Munnich; P Rustin
Journal:  Nat Genet       Date:  1997-10       Impact factor: 38.330

4.  Iron-sulfur protein maturation in human cells: evidence for a function of frataxin.

Authors:  Oliver Stehling; Hans-Peter Elsässer; Bernd Brückel; Ulrich Mühlenhoff; Roland Lill
Journal:  Hum Mol Genet       Date:  2004-10-27       Impact factor: 6.150

5.  Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study.

Authors:  P Rustin; J C von Kleist-Retzow; K Chantrel-Groussard; D Sidi; A Munnich; A Rötig
Journal:  Lancet       Date:  1999-08-07       Impact factor: 79.321

6.  Assembly of human frataxin is a mechanism for detoxifying redox-active iron.

Authors:  Heather A O'Neill; Oleksandr Gakh; Sungjo Park; Jin Cui; Steven M Mooney; Matthew Sampson; Gloria C Ferreira; Grazia Isaya
Journal:  Biochemistry       Date:  2005-01-18       Impact factor: 3.162

7.  Idebenone treatment in Friedreich's ataxia: neurological, cardiac, and biochemical monitoring.

Authors:  G Buyse; L Mertens; G Di Salvo; I Matthijs; F Weidemann; B Eyskens; W Goossens; N Goemans; G R Sutherland; J L K Van Hove
Journal:  Neurology       Date:  2003-05-27       Impact factor: 9.910

8.  Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial.

Authors:  C Mariotti; A Solari; D Torta; L Marano; C Fiorentini; S Di Donato
Journal:  Neurology       Date:  2003-05-27       Impact factor: 9.910

9.  Use of complementary alternative medicine in patients admitted to internal medicine wards.

Authors:  T Azaz-Livshits; M Muszkat; M Levy
Journal:  Int J Clin Pharmacol Ther       Date:  2002-12       Impact factor: 1.366

10.  Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes.

Authors:  M Cossée; A Dürr; M Schmitt; N Dahl; P Trouillas; P Allinson; M Kostrzewa; A Nivelon-Chevallier; K H Gustavson; A Kohlschütter; U Müller; J L Mandel; A Brice; M Koenig; F Cavalcanti; A Tammaro; G De Michele; A Filla; S Cocozza; M Labuda; L Montermini; J Poirier; M Pandolfo
Journal:  Ann Neurol       Date:  1999-02       Impact factor: 10.422

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  15 in total

Review 1.  Milestones in Friedreich ataxia: more than a century and still learning.

Authors:  Agessandro Abrahão; José Luiz Pedroso; Pedro Braga-Neto; Edson Bor-Seng-Shu; Patricia de Carvalho Aguiar; Orlando Graziani Povoas Barsottini
Journal:  Neurogenetics       Date:  2015-02-08       Impact factor: 2.660

Review 2.  Drug development for rare mitochondrial disorders.

Authors:  Orest Hurko
Journal:  Neurotherapeutics       Date:  2013-04       Impact factor: 7.620

Review 3.  Cardiac involvement in hereditary ataxias.

Authors:  Sean Moore; Subha V Raman
Journal:  J Child Neurol       Date:  2012-07-04       Impact factor: 1.987

Review 4.  Transition of Thalassaemia and Friedreich ataxia from fatal to chronic diseases.

Authors:  Annita Kolnagou; Christina N Kontoghiorghe; George J Kontoghiorghes
Journal:  World J Methodol       Date:  2014-12-26

Review 5.  Pharmacotherapy for Friedreich ataxia.

Authors:  Amy Y Tsou; Lisa S Friedman; Robert B Wilson; David R Lynch
Journal:  CNS Drugs       Date:  2009       Impact factor: 5.749

6.  Measuring the rate of progression in Friedreich ataxia: implications for clinical trial design.

Authors:  Lisa S Friedman; Jennifer M Farmer; Susan Perlman; George Wilmot; Christopher M Gomez; Khalaf O Bushara; Katherine D Mathews; S H Subramony; Tetsuo Ashizawa; Laura J Balcer; Robert B Wilson; David R Lynch
Journal:  Mov Disord       Date:  2010-03-15       Impact factor: 10.338

7.  Predictors of progression in patients with Friedreich ataxia.

Authors:  Alison La Pean; Neal Jeffries; Chelsea Grow; Bernard Ravina; Nicholas A Di Prospero
Journal:  Mov Disord       Date:  2008-10-30       Impact factor: 10.338

8.  Childhood ataxia: clinical features, pathogenesis, key unanswered questions, and future directions.

Authors:  Claire N Ashley; Kelly D Hoang; David R Lynch; Susan L Perlman; Bernard L Maria
Journal:  J Child Neurol       Date:  2012-08-01       Impact factor: 1.987

9.  NQO1-dependent redox cycling of idebenone: effects on cellular redox potential and energy levels.

Authors:  Roman H Haefeli; Michael Erb; Anja C Gemperli; Dimitri Robay; Isabelle Courdier Fruh; Corinne Anklin; Robert Dallmann; Nuri Gueven
Journal:  PLoS One       Date:  2011-03-31       Impact factor: 3.240

10.  Redox processes in neurodegenerative disease involving reactive oxygen species.

Authors:  Peter Kovacic; Ratnasamy Somanathan
Journal:  Curr Neuropharmacol       Date:  2012-12       Impact factor: 7.363

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