(Q34654338)

3 August 2017

title

Co-enzyme Q10 and idebenone use in Friedreich's ataxia (English)

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3 August 2017

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ChEBI release 2020-05-28

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2

Jörg B Schulz

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3 August 2017

Michael H Parkinson

1

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3 August 2017

Paola Giunti

series ordinal

3

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3 August 2017

language of work or name

English

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publication date

1 August 2013

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Journal of Neurochemistry

3 August 2017

published in

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3 August 2017

volume

126 Suppl 1

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3 August 2017

page(s)

125-141

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A Strategy for Suppressing Redox Stress within Mitochondria

3 August 2017

cites work

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Friedreich's ataxia: idebenone treatment in early stage patients

21 January 2018

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Cerebrospinal fluid concentrations of idebenone in Friedreich ataxia patients

21 January 2018

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Distribution and breakdown of labeled coenzyme Q10 in rat.

21 January 2018

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Coenzyme Q--biosynthesis and functions

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Plasma coenzyme Q10 response to oral ingestion of coenzyme Q10 formulations

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Intermediate-dose idebenone and quality of life in Friedreich ataxia

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

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Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

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Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes

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Coenzyme Q10 and vitamin E deficiency in Friedreich's ataxia: predictor of efficacy of vitamin E and coenzyme Q10 therapy.

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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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Exercise capacity and idebenone intervention in children and adolescents with Friedreich ataxia

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Clinical and genetic abnormalities in patients with Friedreich's ataxia

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Mammalian pyrimidine biosynthesis: fresh insights into an ancient pathway

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Effects of alkyl side chain modification of coenzyme Q10 on mitochondrial respiratory chain function and cytoprotection

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The mitochondria-targeted anti-oxidant mitoquinone decreases liver damage in a phase II study of hepatitis C patients

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Mitochondrial DNA haplogroups influence the Friedreich's ataxia phenotype

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Impact of Friedreich's Ataxia on health-care resource utilization in the United Kingdom and Germany

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Ubiquinone: cholesterol's reclusive cousin

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Antioxidant treatment of patients with Friedreich ataxia: four-year follow-up

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Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

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Identification and sizing of GAA trinucleotide repeat expansion, investigation for D-loop variations and mitochondrial deletions in Iranian patients with Friedreich's ataxia.

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Coenzyme Q10: absorption, antioxidative properties, determinants, and plasma levels

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Effects of coenzyme Q(10) administration on its tissue concentrations, mitochondrial oxidant generation, and oxidative stress in the rat.

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Cerebellar ataxia and coenzyme Q10 deficiency

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The role of Coenzyme Q in mitochondrial electron transport

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Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia

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A phase 3, double-blind, placebo-controlled trial of idebenone in friedreich ataxia

21 January 2018

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

21 January 2018

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Coenzyme Q10 administration increases brain mitochondrial concentrations and exerts neuroprotective effects

21 January 2018

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Idebenone: an emerging therapy for Friedreich ataxia

21 January 2018

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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

21 January 2018

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Targeting mitochondrial dysfunction and neurodegeneration by means of coenzyme Q10 and its analogues

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

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Glutathione in blood of patients with Friedreich's ataxia.

21 January 2018

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Idebenone treatment in paediatric and adult patients with Friedreich ataxia: long-term follow-up

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Neurological, cardiological, and oculomotor progression in 104 patients with Friedreich ataxia during long-term follow-up

21 January 2018

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia

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

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Idebenone in patients with Friedreich ataxia

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Oxidative stress in patients with Friedreich ataxia.

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Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia

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Dose ranging and efficacy study of high-dose coenzyme Q10 formulations in Huntington's disease mice

21 January 2018

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A double-blind, placebo-controlled study to assess the mitochondria-targeted antioxidant MitoQ as a disease-modifying therapy in Parkinson's disease

21 January 2018

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Effects of idebenone (CV-2619) and its metabolites on respiratory activity and lipid peroxidation in brain mitochondria from rats and dogs.

21 January 2018

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Disposition of idebenone (CV-2619), a new cerebral metabolism improving agent, in rats and dogs.

21 January 2018

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Combined therapy with idebenone and deferiprone in patients with Friedreich's ataxia.

21 January 2018

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The heart in Friedreich ataxia: definition of cardiomyopathy, disease severity, and correlation with neurological symptoms.

21 January 2018

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Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue

21 January 2018

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The Interaction of Q Analogs, Particularly Hydroxydecyl Benzoquinone (Idebenone), with the Respiratory Complexes of Heart Mitochondria

21 January 2018

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

21 January 2018

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Safety, Tolerability, and Pharmacokinetics of High-Dose Idebenone in Patients With Friedreich Ataxia

21 January 2018

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21 January 2018

Identifiers

DOI

10.1111/JNC.12322

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3 August 2017

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