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Times Cited: 148
Times Cited: 148
Times Cited
Times Co-cited
Similarity
Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion.
V Campuzano, L Montermini, M D Moltò, L Pianese, M Cossée, F Cavalcanti, E Monros, F Rodius, F Duclos, A Monticelli,[...]. Science 1996
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Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial.
C Mariotti, A Solari, D Torta, L Marano, C Fiorentini, S Di Donato. Neurology 2003
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Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia.
A O Hausse, Y Aggoun, D Bonnet, D Sidi, A Munnich, A Rötig, P Rustin. Heart 2002
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Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study.
P Rustin, J C von Kleist-Retzow, K Chantrel-Groussard, D Sidi, A Munnich, A Rötig. Lancet 1999
P Rustin, J C von Kleist-Retzow, K Chantrel-Groussard, D Sidi, A Munnich, A Rötig. Lancet 1999
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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia.
A Rötig, P de Lonlay, D Chretien, F Foury, M Koenig, D Sidi, A Munnich, P Rustin. Nat Genet 1997
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Antioxidant treatment of patients with Friedreich ataxia: four-year follow-up.
Paul E Hart, Raffaele Lodi, Bheeshma Rajagopalan, Jane L Bradley, Jenifer G Crilley, Christopher Turner, Andrew M Blamire, David Manners, Peter Styles, Anthony H V Schapira,[...]. Arch Neurol 2005
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Clinical and genetic abnormalities in patients with Friedreich's ataxia.
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Idebenone treatment in Friedreich's ataxia: neurological, cardiac, and biochemical monitoring.
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Oxidative stress in patients with Friedreich ataxia.
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Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia.
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Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes.
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Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits.
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Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial.
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Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria.
F Foury, O Cazzalini. FEBS Lett 1997
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The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis.
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Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes.
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A phase 3, double-blind, placebo-controlled trial of idebenone in friedreich ataxia.
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Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin.
H Koutnikova, V Campuzano, F Foury, P Dollé, O Cazzalini, M Koenig. Nat Genet 1997
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Clinical, biochemical and molecular genetic correlations in Friedreich's ataxia.
J L Bradley, J C Blake, S Chamberlain, P K Thomas, J M Cooper, A H Schapira. Hum Mol Genet 2000
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Increased levels of plasma malondialdehyde in Friedreich ataxia.
M Emond, G Lepage, M Vanasse, M Pandolfo. Neurology 2000
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Idebenone in Friedreich ataxia cardiomyopathy-results from a 6-month phase III study (IONIA).
Sarah J Lagedrost, Martin St John Sutton, Meryl S Cohen, Gary M Satou, Beth D Kaufman, Susan L Perlman, Christian Rummey, Thomas Meier, David R Lynch. Am Heart J 2011
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Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue.
R B Wilson, D M Roof. Nat Genet 1997
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Idebenone treatment in paediatric and adult patients with Friedreich ataxia: long-term follow-up.
Mercè Pineda, Javier Arpa, Raquel Montero, Asunción Aracil, Francisco Domínguez, Marta Galván, Anna Mas, Loreto Martorell, Cristina Sierra, Nuria Brandi,[...]. Eur J Paediatr Neurol 2008
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Coenzyme Q10 and vitamin E deficiency in Friedreich's ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy.
J M Cooper, L V P Korlipara, P E Hart, J L Bradley, A H V Schapira. Eur J Neurol 2008
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Friedreich's ataxia: idebenone treatment in early stage patients.
R Artuch, A Aracil, A Mas, C Colomé, M Rissech, E Monrós, M Pineda. Neuropediatrics 2002
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International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. The Ataxia Neuropharmacology Committee of the World Federation of Neurology.
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Selective iron chelation in Friedreich ataxia: biologic and clinical implications.
Nathalie Boddaert, Kim Hanh Le Quan Sang, Agnès Rötig, Anne Leroy-Willig, Serge Gallet, Francis Brunelle, Daniel Sidi, Jean-Christophe Thalabard, Arnold Munnich, Z Ioav Cabantchik. Blood 2007
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Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.
David Herman, Kai Jenssen, Ryan Burnett, Elisabetta Soragni, Susan L Perlman, Joel M Gottesfeld. Nat Chem Biol 2006
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The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins.
Ulrich Mühlenhoff, Nadine Richhardt, Michael Ristow, Gyula Kispal, Roland Lill. Hum Mol Genet 2002
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Friedreich's ataxia: clinical pilot trial with recombinant human erythropoietin.
Sylvia Boesch, Brigitte Sturm, Sascha Hering, Hans Goldenberg, Werner Poewe, Barbara Scheiber-Mojdehkar. Ann Neurol 2007
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Combined therapy with idebenone and deferiprone in patients with Friedreich's ataxia.
Daniel Velasco-Sánchez, Asuncion Aracil, Raquel Montero, Ana Mas, Lorenzo Jiménez, Mar O'Callaghan, Maria Tondo, Antoni Capdevila, Josep Blanch, Rafael Artuch,[...]. Cerebellum 2011
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The yeast frataxin homologue mediates mitochondrial iron efflux. Evidence for a mitochondrial iron cycle.
D C Radisky, M C Babcock, J Kaplan. J Biol Chem 1999
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Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation.
M Cossée, H Puccio, A Gansmuller, H Koutnikova, A Dierich, M LeMeur, K Fischbeck, P Dollé, M Koenig. Hum Mol Genet 2000
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Cardiac energetics are abnormal in Friedreich ataxia patients in the absence of cardiac dysfunction and hypertrophy: an in vivo 31P magnetic resonance spectroscopy study.
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The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia.
A Filla, G De Michele, F Cavalcanti, L Pianese, A Monticelli, G Campanella, S Cocozza. Am J Hum Genet 1996
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Recombinant human erythropoietin: effects on frataxin expression in vitro.
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Neurological effects of recombinant human erythropoietin in Friedreich's ataxia: a clinical pilot trial.
Sylvia Boesch, Brigitte Sturm, Sascha Hering, Barbara Scheiber-Mojdehkar, Hannes Steinkellner, Hans Goldenberg, Werner Poewe. Mov Disord 2008
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Assessment of neurological efficacy of idebenone in pediatric patients with Friedreich's ataxia: data from a 6-month controlled study followed by a 12-month open-label extension study.
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Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia.
Pierre Rustin, Agnès Rötig, Arnold Munnich, Daniel Sidi. Free Radic Res 2002
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Disabled early recruitment of antioxidant defenses in Friedreich's ataxia.
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Idebenone in patients with Friedreich ataxia.
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Antioxidant enzymes in blood of patients with Friedreich's ataxia.
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Mitochondria-targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants.
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Effects of coenzyme Q10 in early Parkinson disease: evidence of slowing of the functional decline.
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Glutathione in blood of patients with Friedreich's ataxia.
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Mortality in Friedreich ataxia.
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Friedreich's ataxia: iron chelators that target the mitochondrion as a therapeutic strategy?
D R Richardson. Expert Opin Investig Drugs 2003
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The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure.
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Co-cited is the co-citation frequency, indicating how many articles cite the article together with the query article. Similarity is the co-citation as percentage of the times cited of the query article or the article in the search results, whichever is the lowest. These numbers are calculated for the last 100 citations when articles are cited more than 100 times.