(Q56992527)

Expert Opinion on Pharmacotherapy

ChEBI release 2020-05-28

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Caterina Tonon

1

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Raffaele Lodi

object named as

Raffaele Lodi

series ordinal

2

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author name string

Caterina Tonon

series ordinal

1

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Raffaele Lodi

series ordinal

2

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language of work or name

English

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publication date

September 2008

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19 August 2008

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published in

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volume

9

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issue

13

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page(s)

2327-37

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2327-2337

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cites work

Evolution of the Friedreich's ataxia trinucleotide repeat expansion: founder effect and premutations

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Clinical and genetic abnormalities in patients with Friedreich's ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

The correlation of clinical phenotype in Friedreich ataxia with the site of point mutations in the FRDA gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Friedreich's ataxia. Revision of the phenotype according to molecular genetics

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Phenotype correlation and intergenerational dynamics of the Friedreich ataxia GAA trinucleotide repeat

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Chemistry, toxicology, pharmacology and pharmacokinetics of idebenone: a review

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Idebenone. A review of its pharmacodynamic and pharmacokinetic properties, and therapeutic use in age-related cognitive disorders

21 January 2018

1 reference

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Antioxidant properties of 2,3-dimethoxy-5-methyl-6-(10-hydroxydecyl)-1,4-benzoquinone (idebenone).

21 January 2018

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Clinical Pharmacokinetics of Drugs for Alzheimer's Disease

21 January 2018

1 reference

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Inter-rater reliability of the International Cooperative Ataxia Rating Scale (ICARS).

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

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Brain structural damage in Friedreich's ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Brain white matter tracts degeneration in Friedreich ataxia. An in vivo MRI study using tract-based spatial statistics and voxel-based morphometry.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Cardiac energetics are abnormal in Friedreich ataxia patients in the absence of cardiac dysfunction and hypertrophy: an in vivo 31P magnetic resonance spectroscopy study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Phosphorus-31 two-dimensional chemical shift imaging in the myocardium of patients with late onset of Friedreich ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Cerebrospinal fluid concentrations of idebenone in Friedreich ataxia patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Urinary 8-hydroxy-2'-deoxyguanosine as a biological marker of in vivo oxidative DNA damage

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Friedreich's ataxia: idebenone treatment in early stage patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Idebenone in patients with Friedreich ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Oxidative stress, mitochondrial dysfunction and cellular stress response in Friedreich's ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Do idebenone and vitamin therapy shorten the time to achieve visual recovery in Leber hereditary optic neuropathy?

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Long-term treatment with idebenone and riboflavin in a patient with MELAS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Leber's Hereditary Optic Neuropathy (LHON) with 14484/ND6 mutation in a North African patient.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Clinical and brain bioenergetics improvement with idebenone in a patient with Leber's hereditary optic neuropathy: a clinical and 31P-MRS study

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Depressive disorder due to mitochondrial transfer RNALeu(UUR) mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Remission of Leber's hereditary optic neuropathy with idebenone

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

How is disease progress in Friedreich's ataxia best measured? A study of four rating scales.

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Antioxidant use in Friedreich ataxia

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

Early-onset ataxia with cardiomyopathy and retained tendon reflexes maps to the friedreich's ataxia locus on chromosome 9q

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1517%2F14656566.9.13.2327