PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia (Q33552305)

28 July 2017

title

PGC-1alpha down-regulation affects the antioxidant response in Friedreich's ataxia (English)

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28 July 2017

1 reference

Fabio Acquaviva

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Daniele Marmolino

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28 July 2017

Mario Manto

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28 July 2017

Paola Vergara

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28 July 2017

Ajay Ravella

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28 July 2017

Antonella Monticelli

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28 July 2017

Massimo Pandolfo

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28 July 2017

language of work or name

English

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publication date

7 April 2010

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28 July 2017

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28 July 2017

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28 July 2017

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28 July 2017

page(s)

e10025

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Creative Commons Attribution 4.0 International

28 July 2017

start time

7 April 2010

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April 2022 Public Data File from Crossref

copyrighted

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cites work

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Functional genomic analysis of frataxin deficiency reveals tissue-specific alterations and identifies the PPARgamma pathway as a therapeutic target in Friedreich's ataxia

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Impaired nuclear Nrf2 translocation undermines the oxidative stress response in Friedreich ataxia

21 June 2018

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PPAR-gamma agonist Azelaoyl PAF increases frataxin protein and mRNA expression: new implications for the Friedreich's ataxia therapy.

21 June 2018

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AMP-activated protein kinase-regulated activation of the PGC-1alpha promoter in skeletal muscle cells

21 June 2018

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Nrf2-induced antioxidant protection: a promising target to counteract ROS-mediated damage in neurodegenerative disease?

21 June 2018

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RETRACTED: Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype

21 June 2018

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HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model

21 June 2018

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Dynamic regulation of PGC-1alpha localization and turnover implicates mitochondrial adaptation in calorie restriction and the stress response

21 June 2018

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Metallosensors, the ups and downs of gene regulation

21 June 2018

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

21 June 2018

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

21 June 2018

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Role of mitochondria in non-alcoholic fatty liver disease

21 June 2018

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

21 June 2018

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

21 June 2018

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Peroxisome proliferator-activated receptor gamma coactivator 1 coactivators, energy homeostasis, and metabolism

21 June 2018

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

21 June 2018

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GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology

21 June 2018

1 reference

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The role of iron regulatory proteins in mammalian iron homeostasis and disease

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Monomeric yeast frataxin is an iron-binding protein

21 June 2018

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Friedreich's ataxia: from disease mechanisms to therapeutic interventions

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

PGC-1alpha regulates the mitochondrial antioxidant defense system in vascular endothelial cells

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Erralpha and Gabpa/b specify PGC-1alpha-dependent oxidative phosphorylation gene expression that is altered in diabetic muscle

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Structure of frataxin iron cores: an X-ray absorption spectroscopic study

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Bioenergetic analysis of peroxisome proliferator-activated receptor gamma coactivators 1alpha and 1beta (PGC-1alpha and PGC-1beta) in muscle cells

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Regulation and co-ordination of nuclear gene expression during mitochondrial biogenesis

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Frataxin deficiency enhances apoptosis in cells differentiating into neuroectoderm

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Oxidative stress in patients with Friedreich ataxia.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Secondary abnormalities of mitochondrial DNA associated with neurodegeneration

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

A tissue-specific coactivator of steroid receptors, identified in a functional genetic screen

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Friedreich ataxia: an overview

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Transcriptional activation of adipogenesis

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Human peroxisome proliferator activated receptor gamma coactivator 1 (PPARGC1) gene: cDNA sequence, genomic organization, chromosomal localization, and tissue expression

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Cellular energy utilization and molecular origin of standard metabolic rate in mammals

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Glucose metabolism alterations in Friedreich's ataxia

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Glucose and insulin metabolism in Friedreich's ataxia.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

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

21 June 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0010025

Iron binding and oxidation kinetics in frataxin CyaY of Escherichia coli.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0010025

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/10.1371%2FJOURNAL.PONE.0010025

Decreased expression of genes involved in sulfur amino acid metabolism in frataxin-deficient cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0010025

The phylogenetic distribution of frataxin indicates a role in iron-sulfur cluster protein assembly

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0010025

Glutathione in blood of patients with Friedreich's ataxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0010025

Reduction in frataxin causes progressive accumulation of mitochondrial damage.

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0010025

Transcriptional coactivator PGC-1 alpha controls the energy state and contractile function of cardiac muscle

21 January 2018

1 reference

https://api.crossref.org/works/10.1371%2FJOURNAL.PONE.0010025

PGC-1alpha is required for training-induced prevention of age-associated decline in mitochondrial enzymes in mouse skeletal muscle

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

Activation of AMP-activated protein kinase reduces hyperglycemia-induced mitochondrial reactive oxygen species production and promotes mitochondrial biogenesis in human umbilical vein endothelial cells

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2850922

31 October 2018

1 reference

12 December 2020

Accuracy of clinical diagnostic criteria for Friedreich's ataxia

1 reference

12 December 2020

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

1 reference

12 December 2020

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

1 reference

12 December 2020

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

1 reference

12 December 2020

Rescue of the Friedreich's ataxia knockout mouse by human YAC transgenesis

1 reference

12 December 2020

Transcriptional control of energy homeostasis through the PGC1 coactivators

1 reference

12 December 2020

Increased levels of plasma malondialdehyde in Friedreich ataxia

1 reference

12 December 2020

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

1 reference

12 December 2020

10.1371/JOURNAL.PONE.0010025

Identifiers

DOI

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28 July 2017

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28 July 2017

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