(Q37376101)

20 August 2017

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title

Friedreich's Ataxia: from the (GAA)n repeat mediated silencing to new promising molecules for therapy (English)

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20 August 2017

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Daniele Marmolino

1

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20 August 2017

Fabio Acquaviva

series ordinal

2

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20 August 2017

publication date

23 January 2009

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20 August 2017

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20 August 2017

volume

8

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20 August 2017

issue

3

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20 August 2017

page(s)

245-259

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Glucose metabolism alterations in Friedreich's ataxia

20 August 2017

cites work

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https://api.crossref.org/works/10.1007%2FS12311-008-0084-2

Late-onset Friedreich's ataxia. Molecular genetics, clinical neurophysiology, and magnetic resonance imaging

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS12311-008-0084-2

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/10.1007%2FS12311-008-0084-2

Familial cerebellar ataxia with muscle coenzyme Q10 deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS12311-008-0084-2

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS12311-008-0084-2

Development of potential iron chelators for the treatment of Friedreich's ataxia: ligands that mobilize mitochondrial iron

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS12311-008-0084-2

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS12311-008-0084-2

N-terminal iron-mediated self-cleavage of human frataxin: regulation of iron binding and complex formation with target proteins

21 January 2018

1 reference

12 December 2020

Targeting proteins to mitochondria using TAT.

1 reference

12 December 2020

In vivo maturation of human frataxin

1 reference

12 December 2020

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12 December 2020

Identification and sizing of the GAA trinucleotide repeat expansion of Friedreich's ataxia in 56 patients. Clinical and genetic correlates

1 reference

12 December 2020

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

1 reference

12 December 2020

Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators

1 reference

12 December 2020

The FET3 gene of S. cerevisiae encodes a multicopper oxidase required for ferrous iron uptake

1 reference

12 December 2020

Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

1 reference

12 December 2020

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

1 reference

12 December 2020

Iron and Friedreich ataxia.

1 reference

12 December 2020

Clinical Description and Roentgenologic Evaluation of Patients with Friedreich's Ataxia

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12 December 2020

Frataxin interacts functionally with mitochondrial electron transport chain proteins

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12 December 2020

Real time PCR quantification of frataxin mRNA in the peripheral blood leucocytes of Friedreich ataxia patients and carriers.

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12 December 2020

Friedreich's ataxia: iron chelators that target the mitochondrion as a therapeutic strategy?

1 reference

12 December 2020

Relationship between histone H3 lysine 9 methylation, transcription repression, and heterochromatin protein 1 recruitment.

1 reference

12 December 2020

Clinical development of histone deacetylase inhibitors as anticancer agents

1 reference

12 December 2020

Frataxin and mitochondrial carrier proteins, Mrs3p and Mrs4p, cooperate in providing iron for heme synthesis

1 reference

12 December 2020

HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model

1 reference

12 December 2020

Pioglitazone for type 2 diabetes mellitus

1 reference

12 December 2020

DNA triplexes and Friedreich ataxia

1 reference

12 December 2020

Rosiglitazone for type 2 diabetes mellitus

1 reference

12 December 2020

Accuracy of clinical diagnostic criteria for Friedreich's ataxia

1 reference

12 December 2020

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

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12 December 2020

Increased iron in the dentate nucleus of patients with Friedrich's ataxia

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12 December 2020

The structures of frataxin oligomers reveal the mechanism for the delivery and detoxification of iron.

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12 December 2020

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

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12 December 2020

The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues

1 reference

12 December 2020

Erythropoietin prevents neuronal apoptosis after cerebral ischemia and metabolic stress

1 reference

12 December 2020

A permease-oxidase complex involved in high-affinity iron uptake in yeast

1 reference

12 December 2020

Fragile-X syndrome and myotonic dystrophy: parallels and paradoxes

1 reference

12 December 2020

Cryptic proteolytic activity of dihydrolipoamide dehydrogenase

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12 December 2020

The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells

1 reference

12 December 2020

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

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12 December 2020

Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis

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12 December 2020

Glucose and insulin metabolism in Friedreich's ataxia.

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12 December 2020

A persistent RNA.DNA hybrid formed by transcription of the Friedreich ataxia triplet repeat in live bacteria, and by T7 RNAP in vitro

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12 December 2020

DNA methylation in intron 1 of the frataxin gene is related to GAA repeat length and age of onset in Friedreich ataxia patients.

1 reference

12 December 2020

Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features

1 reference

12 December 2020

Frataxin knockin mouse

1 reference

12 December 2020

Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription

1 reference

12 December 2020

Erythropoietin receptor is expressed in rat hippocampal and cerebral cortical neurons, and erythropoietin prevents in vitro glutamate-induced neuronal death

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12 December 2020

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

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12 December 2020

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

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12 December 2020

Trinucleotide Repeat Disorders

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12 December 2020

Randomised controlled trial of vitamin E in patients with coronary disease: Cambridge Heart Antioxidant Study (CHAOS)

1 reference

12 December 2020

Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae

1 reference

12 December 2020

Fragile X syndrome and Friedreich's ataxia: two different paradigms for repeat induced transcript insufficiency

1 reference

12 December 2020

Repeat-induced epigenetic changes in intron 1 of the frataxin gene and its consequences in Friedreich ataxia

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12 December 2020

Clinical and genetic abnormalities in patients with Friedreich's ataxia

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12 December 2020

A novel TAT-mitochondrial signal sequence fusion protein is processed, stays in mitochondria, and crosses the placenta

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12 December 2020

Antisense transcription and heterochromatin at the DM1 CTG repeats are constrained by CTCF.

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12 December 2020

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

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12 December 2020

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

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12 December 2020

PGC-1alpha-responsive genes involved in oxidative phosphorylation are coordinately downregulated in human diabetes

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12 December 2020

Physical evidence that yeast frataxin is an iron storage protein

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12 December 2020

Therapeutics development for triplet repeat expansion diseases

1 reference

12 December 2020

The role of histone acetylation in SMN gene expression.

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12 December 2020

The pharmacokinetics of erythropoietin in the cerebrospinal fluid after intravenous administration of recombinant human erythropoietin

1 reference

12 December 2020

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

1 reference

12 December 2020

The mitochondrial protein frataxin prevents nuclear damage

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12 December 2020

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

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12 December 2020

Upregulation of expression from the FRDA genomic locus for the therapy of Friedreich ataxia

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12 December 2020

The evolution of iron chelators for the treatment of iron overload disease and cancer.

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12 December 2020

Small molecules affecting transcription in Friedreich ataxia

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12 December 2020

Recombinant human erythropoietin increases frataxin protein expression without increasing mRNA expression

1 reference

12 December 2020

Friedreich's ataxia: clinical pilot trial with recombinant human erythropoietin

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12 December 2020

A pool of extramitochondrial frataxin that promotes cell survival

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12 December 2020

In vivo evidence that erythropoietin protects neurons from ischemic damage

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12 December 2020

The GAA triplet-repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure

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12 December 2020

Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia

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12 December 2020

Friedreich's ataxia: coenzyme Q10 and vitamin E therapy

1 reference

12 December 2020

PGC-1alpha: a potent transcriptional cofactor involved in the pathogenesis of type 2 diabetes

1 reference

12 December 2020

DNA methylation and histone deacetylation in the control of gene expression: basic biochemistry to human development and disease.

1 reference

12 December 2020

Reduced risk of colon cancer with high intake of vitamin E: the Iowa Women's Health Study

1 reference

12 December 2020

DNA triplet repeats mediate heterochromatin-protein-1-sensitive variegated gene silencing

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12 December 2020

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

1 reference

12 December 2020

Atypical Friedreich ataxia caused by compound heterozygosity for a novel missense mutation and the GAA triplet-repeat expansion

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12 December 2020

Genetic data and natural history of Friedreich's disease: a study of 80 Italian patients

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12 December 2020

Deferiprone targets aconitase: implication for Friedreich's ataxia treatment

1 reference

12 December 2020

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

12 December 2020

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

1 reference

12 December 2020

Recombinant human erythropoietin: effects on frataxin expression in vitro

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12 December 2020

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

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12 December 2020

Redistribution of accumulated cell iron: a modality of chelation with therapeutic implications

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12 December 2020

Transcriptional regulatory circuits controlling mitochondrial biogenesis and function

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12 December 2020

Mechanisms controlling mitochondrial biogenesis and respiration through the thermogenic coactivator PGC-1

1 reference

12 December 2020

DATATOP: a decade of neuroprotective inquiry. Parkinson Study Group. Deprenyl And Tocopherol Antioxidative Therapy Of Parkinsonism.

1 reference

12 December 2020

Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia

1 reference

12 December 2020

An update on the cardiac effects of erythropoietin cardioprotection by erythropoietin and the lessons learnt from studies in neuroprotection.

1 reference

12 December 2020

Iron dysregulation in Friedreich ataxia

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12 December 2020

Delivery of bioactive molecules to mitochondria in vivo.

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12 December 2020

Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells.

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12 December 2020

The transcriptional co-activator p/CIP binds CBP and mediates nuclear-receptor function

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12 December 2020

Reduction in frataxin causes progressive accumulation of mitochondrial damage.

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12 December 2020

Frataxin, iron-sulfur clusters, heme, ROS, and aging.

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12 December 2020

Remodeling chromatin and stress resistance in the central nervous system: histone deacetylase inhibitors as novel and broadly effective neuroprotective agents.

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12 December 2020

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

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12 December 2020

Inhibitory effects of expanded GAA.TTC triplet repeats from intron I of the Friedreich ataxia gene on transcription and replication in vivo

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12 December 2020

Selective iron chelation in Friedreich ataxia: biologic and clinical implications

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12 December 2020

Components involved in assembly and dislocation of iron-sulfur clusters on the scaffold protein Isu1p

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12 December 2020

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

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12 December 2020

Drug insight: effects mediated by peroxisome proliferator-activated receptor-gamma in CNS disorders

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12 December 2020

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

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12 December 2020

Iron-dependent self-assembly of recombinant yeast frataxin: implications for Friedreich ataxia

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12 December 2020

Friedreich ataxia: an overview

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12 December 2020

Coordinated reduction of genes of oxidative metabolism in humans with insulin resistance and diabetes: Potential role of PGC1 and NRF1

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12 December 2020

Alleviating transcript insufficiency caused by Friedreich's ataxia triplet repeats

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12 December 2020

Heterochromatin: silence is golden.

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12 December 2020

Extra-mitochondrial localisation of frataxin and its association with IscU1 during enterocyte-like differentiation of the human colon adenocarcinoma cell line Caco-2.

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12 December 2020

Locus heterogeneity in Friedreich ataxia

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12 December 2020

Erythropoietin crosses the blood-brain barrier to protect against experimental brain injury

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12 December 2020

Selective targeting of a redox-active ubiquinone to mitochondria within cells: antioxidant and antiapoptotic properties

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12 December 2020

Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different rates

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12 December 2020

Peroxisome proliferator-activated receptors: regulation of transcriptional activities and roles in inflammation

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12 December 2020

Supramolecular assemblies of human frataxin are formed via subunit-subunit interactions mediated by a non-conserved amino-terminal region

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12 December 2020

Yeast Frataxin Sequentially Chaperones and Stores Iron by Coupling Protein Assembly with Iron Oxidation

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12 December 2020

10.1007/S12311-008-0084-2

Identifiers

DOI

1 reference

release_k4bkvd76kjdkrgl7om5qssyv44

20 August 2017

Fatcat ID

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Fatcat

https://api.fatcat.wiki/v0/release/k4bkvd76kjdkrgl7om5qssyv44

24 November 2022

mapped directly with Wikidata item

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