Drug Insight: antioxidant therapy in inherited ataxias (Q37078851)

18 August 2017

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title

Drug Insight: antioxidant therapy in inherited ataxias (English)

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18 August 2017

Massimo Pandolfo

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18 August 2017

1 February 2008

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Nature Clinical Practice Neurology

18 August 2017

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18 August 2017

4

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18 August 2017

86-96

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18 August 2017

2

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Measuring Friedreich ataxia: Interrater reliability of a neurologic rating scale.

18 August 2017

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

Idebenone treatment fails to slow cognitive decline in Alzheimer's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

Oxidative stress in patients with Friedreich ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

Redox regulation of cellular stress response in aging and neurodegenerative disorders: role of vitagenes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Redox modulation of heat shock protein expression by acetylcarnitine in aging brain: relationship to antioxidant status and mitochondrial function.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Sticky DNA: self-association properties of long GAA.TTC repeats in R.R.Y triplex structures from Friedreich's ataxia.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

The yeast frataxin homologue mediates mitochondrial iron efflux. Evidence for a mitochondrial iron cycle

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Transcription of the yeast iron regulon does not respond directly to iron but rather to iron-sulfur cluster biosynthesis.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Oxidative stress and protease dysfunction in the yeast model of Friedreich ataxia

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Up-regulation of c-Jun N-terminal kinase pathway in Friedreich's ataxia cells

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Manganese superoxide dismutase induction by iron is impaired in Friedreich ataxia cells

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Increased levels of plasma malondialdehyde in Friedreich ataxia

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Antioxidant enzymes in blood of patients with Friedreich's ataxia.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Friedreich ataxia: the oxidative stress paradox

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Scale for the assessment and rating of ataxia: development of a new clinical scale

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Therapeutic potential of N-acetylcysteine in age-related mitochondrial neurodegenerative diseases

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Stimulation of the respiratory and phosphorylating activities in rat brain mitochondria by idebenone (CV-2619), a new agent improving cerebral metabolism.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Quinone analogues regulate mitochondrial substrate competitive oxidation

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

A controlled study of 2 doses of idebenone in the treatment of Alzheimer's disease.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

A controlled trial of idebenone in Huntington's disease

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Cerebrospinal fluid concentrations of idebenone in Friedreich ataxia patients

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a randomised, placebo-controlled trial

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

DNA single-strand break repair is impaired in aprataxin-related ataxia.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Senataxin, defective in ataxia oculomotor apraxia type 2, is involved in the defense against oxidative DNA damage

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Impaired genomic stability and increased oxidative stress exacerbate different features of Ataxia-telangiectasia.

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Dramatic extension of tumor latency and correction of neurobehavioral phenotype in Atm-mutant mice with a nitroxide antioxidant

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Effect of the reduction of superoxide dismutase 1 and 2 or treatment with alpha-tocopherol on tumorigenesis in Atm-deficient mice

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Spinocerebellar ataxias: an update

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Cerebellar ataxia with coenzyme Q10 deficiency: Diagnosis and follow-up after coenzyme Q10 supplementation

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https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Friedreich's ataxia: idebenone treatment in early stage patients

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1038%2FNCPNEURO0704

7 January 2021

Identifiers

DOI

10.1038/NCPNEURO0704

1 reference

18 August 2017

1 reference

PubMed ID

18256680