(Q46156695)

20 December 2017

title

Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins (English)

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20 December 2017

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5

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20 December 2017

Anne-Laure Bulteau

2

object named as

Anne-Laure Bulteau

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20 December 2017

Blanche Guillon

1

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20 December 2017

Marie Wattenhofer-Donzé

3

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20 December 2017

Stéphane Schmucker

4

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20 December 2017

Hélène Puccio

6

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20 December 2017

Jean-Claude Drapier

7

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20 December 2017

Cécile Bouton

8

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20 December 2017

language of work or name

English

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publication date

12 January 2008

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20 December 2017

published in

FEBS Journal

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20 December 2017

volume

276

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20 December 2017

issue

4

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20 December 2017

page(s)

1036-1047

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20 December 2017

Friedreich ataxia: a paradigm for mitochondrial diseases

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

21 January 2018

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Conditional mouse models for Friedreich ataxia, a neurodegenerative disorder associating cardiomyopathy

21 January 2018

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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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Inhibition of Fe-S cluster biosynthesis decreases mitochondrial iron export: evidence that Yfh1p affects Fe-S cluster synthesis

21 January 2018

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

21 January 2018

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Mrs3p, Mrs4p, and frataxin provide iron for Fe-S cluster synthesis in mitochondria.

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

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

21 January 2018

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Friedreich ataxia: the oxidative stress paradox

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

21 January 2018

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

21 January 2018

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Binding of yeast frataxin to the scaffold for Fe-S cluster biogenesis, Isu

21 January 2018

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The ubiquitin-proteasome system in cardiac physiology and pathology

21 January 2018

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Protein degradation within mitochondria: versatile activities of AAA proteases and other peptidases

21 January 2018

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Importance of the lon protease in mitochondrial maintenance and the significance of declining lon in aging.

21 January 2018

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Mitochondrial localization and oligomeric structure of HClpP, the human homologue of E. coli ClpP

21 January 2018

1 reference

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Molecular cloning and characterization of a mouse homolog of bacterial ClpX, a novel mammalian class II member of the Hsp100/Clp chaperone family

21 January 2018

1 reference

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Functional proteolytic complexes of the human mitochondrial ATP-dependent protease, hClpXP

21 January 2018

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Proteomic analysis of mitochondrial protein turnover: identification of novel substrate proteins of the matrix protease pim1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism

21 January 2018

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Changes in rat liver mitochondria with aging. Lon protease-like reactivity and N(epsilon)-carboxymethyllysine accumulation in the matrix

21 January 2018

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Reversible redox-dependent modulation of mitochondrial aconitase and proteolytic activity during in vivo cardiac ischemia/reperfusion

21 January 2018

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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

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Protein modification by oxidants and the role of proteolytic enzymes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

The human LON protease binds to mitochondrial promoters in a single-stranded, site-specific, strand-specific manner

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

DNA and RNA binding by the mitochondrial lon protease is regulated by nucleotide and protein substrate

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Roles for the human ATP-dependent Lon protease in mitochondrial DNA maintenance

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Crystal structure of mitochondrial respiratory membrane protein complex II

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

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

21 January 2018

1 reference

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Decreased proteolysis caused by protein aggregates, inclusion bodies, plaques, lipofuscin, ceroid, and 'aggresomes' during oxidative stress, aging, and disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Protein degradation in mitochondria: implications for oxidative stress, aging and disease: a novel etiological classification of mitochondrial proteolytic disorders.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

ClpP: a distinctive family of cylindrical energy-dependent serine proteases

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Human ClpP protease: cDNA sequence, tissue-specific expression and chromosomal assignment of the gene

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

A human mitochondrial ATP-dependent protease that is highly homologous to bacterial Lon protease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Transmission of cell stress from endoplasmic reticulum to mitochondria: enhanced expression of Lon protease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Induction of Zf9 in the kidney following early ischemia/reperfusion

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Expression and distribution of HuR during ATP depletion and recovery in proximal tubule cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

A mitochondrial specific stress response in mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

ATP-dependent proteases that also chaperone protein biogenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Enhanced mitochondrial biogenesis is associated with increased expression of the mitochondrial ATP-dependent Lon protease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Respiratory complex I: mechanistic and structural insights provided by the crystal structure of the hydrophilic domain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

URF6, last unidentified reading frame of human mtDNA, codes for an NADH dehydrogenase subunit

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Chloroplast iron-sulfur cluster protein maturation requires the essential cysteine desulfurase CpNifS

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

Towards the control of intracellular protein turnover: mitochondrial Lon protease inhibitors versus proteasome inhibitors

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1742-4658.2008.06847.X

10.1111/J.1742-4658.2008.06847.X

21 January 2018

Identifiers

DOI

1 reference

20 December 2017

PubMed ID

19154341

1 reference

20 December 2017