(Q33363024)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18725397%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

title

The in vivo mitochondrial two-step maturation of human frataxin (English)

1 reference

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12 January 2020

5

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12 January 2020

author name string

Stéphane Schmucker

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18725397%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

Manuela Argentini

2

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18725397%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

Nadège Carelle-Calmels

3

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18725397%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

Alain Martelli

4

1 reference

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12 January 2020

publication date

25 August 2008

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18725397%20AND%20SRC:MED&resulttype=core&format=json

12 January 2020

published in

Human Molecular Genetics

1 reference

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12 January 2020

volume

17

1 reference

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12 January 2020

issue

22

1 reference

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12 January 2020

page(s)

3521-3531

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18725397%20AND%20SRC:MED&resulttype=core&format=json

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

12 January 2020

cites work

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

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Frataxin activates mitochondrial energy conversion and oxidative phosphorylation

21 January 2018

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

21 January 2018

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

21 January 2018

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A structural approach to understanding the iron-binding properties of phylogenetically different frataxins.

21 January 2018

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Yeast frataxin solution structure, iron binding, and ferrochelatase interaction.

21 January 2018

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Monomeric yeast frataxin is an iron-binding protein

21 January 2018

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

21 January 2018

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

21 January 2018

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Drosophila frataxin: an iron chaperone during cellular Fe-S cluster bioassembly

21 January 2018

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

21 January 2018

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Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia

21 January 2018

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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

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A pool of extramitochondrial frataxin that promotes cell survival

21 January 2018

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Targeted disruption of hepatic frataxin expression causes impaired mitochondrial function, decreased life span and tumor growth in mice

21 January 2018

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Induction of oxidative metabolism by mitochondrial frataxin inhibits cancer growth: Otto Warburg revisited

21 January 2018

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Crystal structure of human frataxin

21 January 2018

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Towards a structural understanding of Friedreich's ataxia: the solution structure of frataxin

21 January 2018

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

21 January 2018

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Recent advances in the molecular pathogenesis of Friedreich ataxia

21 January 2018

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Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDN244

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

21 January 2018

1 reference

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In vivo maturation of human frataxin

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Yeast and human frataxin are processed to mature form in two sequential steps by the mitochondrial processing peptidase.

21 January 2018

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Lateral-flow immunoassay for the frataxin protein in Friedreich's ataxia patients and carriers

21 January 2018

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

21 January 2018

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

21 January 2018

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Cryptic proteolytic activity of dihydrolipoamide dehydrogenase

21 January 2018

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

21 January 2018

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Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer

21 January 2018

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Novel subunits of the TATA binding protein free TAFII-containing transcription complex identified by matrix-assisted laser desorption/ionization-time of flight mass spectrometry following one-dimensional gel electrophoresis

21 January 2018

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Solution Structure of the Bacterial Frataxin Ortholog, CyaY

21 January 2018

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