Consequences of the pathogenic T9176C mutation of human mitochondrial DNA on yeast mitochondrial ATP synthase (Q33936361)

30 July 2017

title

Consequences of the pathogenic T9176C mutation of human mitochondrial DNA on yeast mitochondrial ATP synthase (English)

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30 July 2017

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Marc Blondel

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Vincent Procaccio

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Roza Kucharczyk

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30 July 2017

Nahia Ezkurdia

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30 July 2017

Elodie Couplan

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30 July 2017

Sharon H Ackerman

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30 July 2017

Jean-Paul di Rago

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30 July 2017

language of work or name

English

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publication date

4 January 2010

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Biochimica et Biophysica Acta

30 July 2017

published in

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30 July 2017

volume

1797

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30 July 2017

issue

6-7

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30 July 2017

page(s)

1105-1112

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Introducing the human Leigh syndrome mutation T9176G into Saccharomyces cerevisiae mitochondrial DNA leads to severe defects in the incorporation of Atp6p into the ATP synthase and in the mitochondrial morphology.

30 July 2017

cites work

1 reference

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5 July 2018

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5 July 2018

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The leader peptide of yeast Atp6p is required for efficient interaction with the Atp9p ring of the mitochondrial ATPase

5 July 2018

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A yeast model of the neurogenic ataxia retinitis pigmentosa (NARP) T8993G mutation in the mitochondrial ATP synthase-6 gene

5 July 2018

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The metalloprotease encoded by ATP23 has a dual function in processing and assembly of subunit 6 of mitochondrial ATPase

5 July 2018

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ATP6 homoplasmic mutations inhibit and destabilize the human F1F0-ATP synthase without preventing enzyme assembly and oligomerization.

5 July 2018

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5 July 2018

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Function, structure, and biogenesis of mitochondrial ATP synthase

5 July 2018

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Transmission and prenatal diagnosis of the T9176C mitochondrial DNA mutation

5 July 2018

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Atp10p assists assembly of Atp6p into the F0 unit of the yeast mitochondrial ATPase

5 July 2018

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Mechanics of coupling proton movements to c-ring rotation in ATP synthase

5 July 2018

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Investigation of the role and mechanism of IF1 and STF1 proteins, twin inhibitory peptides which interact with the yeast mitochondrial ATP synthase

5 July 2018

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5 July 2018

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Pathogenesis of primary defects in mitochondrial ATP synthesis.

5 July 2018

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Identification of a nuclear gene (FMC1) required for the assembly/stability of yeast mitochondrial F(1)-ATPase in heat stress conditions

5 July 2018

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Impaired ATP synthase assembly associated with a mutation in the human ATP synthase subunit 6 gene

5 July 2018

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Maintenance and integrity of the mitochondrial genome: a plethora of nuclear genes in the budding yeast

5 July 2018

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The assembly factor Atp11p binds to the beta-subunit of the mitochondrial F(1)-ATPase

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

Oligomycin induces a decrease in the cellular content of a pathogenic mutation in the human mitochondrial ATPase 6 gene.

5 July 2018

1 reference

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Oxidative phosphorylation at the fin de siècle

5 July 2018

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Expression of a recoded nuclear gene inserted into yeast mitochondrial DNA is limited by mRNA-specific translational activation.

5 July 2018

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NH2-terminal sequence of the isolated yeast ATP synthase subunit 6 reveals post-translational cleavage

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

Amino acid substitutions in mitochondrial ATPase subunit 6 of Saccharomyces cerevisiae leading to oligomycin resistance.

5 July 2018

1 reference

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Rhodamine 123 as a probe of transmembrane potential in isolated rat-liver mitochondria: spectral and metabolic properties

5 July 2018

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Characterization of ATP11 and detection of the encoded protein in mitochondria of Saccharomyces cerevisiae

5 July 2018

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Biochemical consequences in yeast of the human mitochondrial DNA 8993T>C mutation in the ATPase6 gene found in NARP/MILS patients

5 July 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

Structural interactions between transmembrane helices 4 and 5 of subunit a and the subunit c ring of Escherichia coli ATP synthase

29 October 2018

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F1-catalysed ATP hydrolysis is required for mitochondrial biogenesis in Saccharomyces cerevisiae growing under conditions where it cannot respire

29 October 2018

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

The T9176G mtDNA mutation severely affects ATP production and results in Leigh syndrome

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

DNA sequence analysis of the Olir2-76 and Ossr1-92 alleles of the Oli-2 region of the yeast Saccharomyces cerevisiae. Analysis of related amino-acid substitutions and protein-antibiotic interaction

29 October 2018

1 reference

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Subcomplexes of human ATP synthase mark mitochondrial biosynthesis disorders

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

Altered properties of mitochondrial ATP-synthase in patients with a T-->G mutation in the ATPase 6 (subunit a) gene at position 8993 of mtDNA

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

Yeast Cells Lacking the Mitochondrial Gene Encoding the ATP Synthase Subunit 6 Exhibit a Selective Loss of Complex IV and Unusual Mitochondrial Morphology

29 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2891117

Genetic transformation of Saccharomyces cerevisiae mitochondria

10 December 2018

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12 December 2020

Level of ATP synthase activity required for yeast Saccharomyces cerevisiae to grow on glycerol media

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12 December 2020

A novel mitochondrial ATPase 6 point mutation in familial bilateral striatal necrosis

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12 December 2020

Identification of functional domains in Atp11p. Protein required for assembly of the mitochondrial F1-ATPase in yeast

1 reference

12 December 2020

Fulminant Leigh syndrome and sudden unexpected death in a family with the T9176C mutation of the mitochondrial ATPase 6 gene

1 reference

12 December 2020

Clinical and biochemical characteristics in patients with a high mutant load of the mitochondrial T8993G/C mutations

1 reference

12 December 2020

10.1016/J.BBABIO.2009.12.022

Identifiers

DOI

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30 July 2017

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30 July 2017

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