Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen (Q33496330)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19703283%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

title

Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen (English)

1 reference

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6 February 2020

1 reference

1

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6 February 2020

Helene Puccio

9

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6 February 2020

author name string

Hervé Seznec

2

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6 February 2020

Pascal Villa

3

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6 February 2020

Laurence Reutenauer

4

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6 February 2020

Marcel Hibert

5

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6 February 2020

Jacques Haiech

6

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6 February 2020

Pierre Rustin

7

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6 February 2020

Michel Koenig

8

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6 February 2020

publication date

24 August 2009

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6 February 2020

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6 February 2020

volume

9

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6 February 2020

page(s)

46

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Cell functions impaired by frataxin deficiency are restored by drug-mediated iron relocation

6 February 2020

cites work

1 reference

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HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model

21 June 2018

1 reference

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Drug Insight: antioxidant therapy in inherited ataxias

21 June 2018

1 reference

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The effects of frataxin silencing in HeLa cells are rescued by the expression of human mitochondrial ferritin

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

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

21 June 2018

1 reference

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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

1 reference

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

21 June 2018

1 reference

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Hemin rescues adrenodoxin, heme a and cytochrome oxidase activity in frataxin-deficient oligodendroglioma cells

21 June 2018

1 reference

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Acidic residues of yeast frataxin have an essential role in Fe-S cluster assembly

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

Frataxin knockdown causes loss of cytoplasmic iron-sulfur cluster functions, redox alterations and induction of heme transcripts

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

Iron-sulfur cluster biosynthesis: characterization of Escherichia coli CYaY as an iron donor for the assembly of [2Fe-2S] clusters in the scaffold IscU.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

Selective optimization of side activities: the SOSA approach

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian 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=2744904

Friedreich's ataxia, no changes in mitochondrial labile iron in human lymphoblasts and fibroblasts: a decrease in antioxidative capacity?

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

Iron-sulfur protein maturation in human cells: evidence for a function 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=2744904

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

A cellular model for Friedreich Ataxia reveals small-molecule glutathione peroxidase mimetics as novel treatment strategy

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

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=2744904

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=2744904

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=2744904

A Simple Statistical Parameter for Use in Evaluation and Validation of High Throughput Screening Assays

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

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=2744904

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

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

21 June 2018

1 reference

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2744904

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

21 June 2018

1 reference

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Inhibition of fibrillin 1 expression using U1 snRNA as a vehicle for the presentation of antisense targeting sequence

21 June 2018

1 reference

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Clinical and genetic abnormalities in patients with Friedreich's ataxia

21 June 2018

1 reference

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An immortalized xeroderma pigmentosum, group C, cell line which replicates SV40 shuttle vectors

21 June 2018

1 reference

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Role of oxidative damage in Friedreich's ataxia

21 June 2018

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Glutathione in blood of patients with Friedreich's ataxia.

21 January 2018

1 reference

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Friedreich ataxia: the oxidative stress paradox

21 January 2018

1 reference

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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

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

A non-essential function for yeast frataxin in iron-sulfur cluster assembly

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

The yeast frataxin homolog Yfh1p plays a specific role in the maturation of cellular Fe/S proteins

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

Mitochondrial frataxin interacts with ISD11 of the NFS1/ISCU complex and multiple mitochondrial chaperones.

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

Selective iron chelation in Friedreich ataxia: biologic and clinical implications

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

Site- and time-specific gene targeting in the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

RNAi-mediated suppression of the mitochondrial iron chaperone, frataxin, in Drosophila

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1186%2F1471-2377-9-46

Delivery of a hammerhead ribozyme specifically down-regulates the production of fibrillin-1 by cultured dermal fibroblasts

21 January 2018

1 reference

12 December 2020

The cardiomyopathy of Friedreich's ataxia morphological observations in 3 cases

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

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

Urinary isoprostanes in Friedreich ataxia: lack of correlation with disease features

1 reference

12 December 2020

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

1 reference

12 December 2020

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia

1 reference

12 December 2020

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

1 reference

12 December 2020

1 reference

12 December 2020

Biochemical and molecular investigations in respiratory chain deficiencies

1 reference

12 December 2020

Increased levels of plasma malondialdehyde in Friedreich ataxia

1 reference

12 December 2020

Identifiers

DOI

10.1186/1471-2377-9-46

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19703283%20AND%20SRC:MED&resulttype=core&format=json

Dimensions Publication ID

6 February 2020

1 reference

https://scigraph.springernature.com/pub.10.1186/1471-2377-9-46

9 September 2019

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:19703283%20AND%20SRC:MED&resulttype=core&format=json

6 February 2020

PubMed publication ID

19703283

1 reference