Impaired ATP synthase assembly associated with a mutation in the human ATP synthase subunit 6 gene (Q31561021)

2 July 2017

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

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2 July 2017

Nijtmans LG

1

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2 July 2017

Henderson NS

series ordinal

2

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2 July 2017

Attardi G

series ordinal

3

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2 July 2017

Holt IJ

series ordinal

4

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2 July 2017

language of work or name

English

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publication date

13 November 2000

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Journal of Biological Chemistry

2 July 2017

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2 July 2017

276

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2 July 2017

9

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2 July 2017

6755-6762

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Structure at 2.8 A resolution of F1-ATPase from bovine heart mitochondria

2 July 2017

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Energy transduction in ATP synthase

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

The mitochondrial DNA mutation at 8993 associated with NARP slows the rate of ATP synthesis in isolated lymphoblast mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Cytoplasmic transfer of the mtDNA nt 8993 T-->G (ATP6) point mutation associated with Leigh syndrome into mtDNA-less cells demonstrates cosegregation with a decrease in state III respiration and ADP/O ratio

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Analysis of oxidative phosphorylation complexes in cultured human fibroblasts and amniocytes by blue-native-electrophoresis using mitoplasts isolated with the help of digitonin.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Blue native electrophoresis for isolation of membrane protein complexes in enzymatically active form

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Analysis of molecular masses and oligomeric states of protein complexes by blue native electrophoresis and isolation of membrane protein complexes by two-dimensional native electrophoresis

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Assembly of cytochrome-c oxidase in cultured human cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Quantification of muscle mitochondrial oxidative phosphorylation enzymes via histochemical staining of blue native polyacrylamide gels.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Rapid colorimetric assay for cellular growth and survival: Application to proliferation and cytotoxicity assays

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

In vitro genetic transfer of protein synthesis and respiration defects to mitochondrial DNA-less cells with myopathy-patient mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Molecular architecture of the rotary motor in ATP synthase

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Functional F1-ATPase essential in maintaining growth and membrane potential of human mitochondrial DNA-depleted rho degrees cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Radiolabeling of natural adenosine triphosphatase inhibitor with phenyl (14C)isothiocyanate and study of its interaction with mitochondrial adenosine triphosphatase. Localization of inhibitor binding sites and stoichiometry of binding

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Defects in mitochondrial protein synthesis and respiratory chain activity segregate with the tRNA(Leu(UUR)) mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Introduction of disease-related mitochondrial DNA deletions into HeLa cells lacking mitochondrial DNA results in mitochondrial dysfunction

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Different cellular backgrounds confer a marked advantage to either mutant or wild-type mitochondrial genomes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Structure, functioning, and assembly of the ATP synthase in cells from patients with the T8993G mitochondrial DNA mutation. Comparison with the enzyme in Rho(0) cells completely lacking mtdna

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

Catalytic activities of mitochondrial ATP synthase in patients with mitochondrial DNA T8993G mutation in the ATPase 6 gene encoding subunit a

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M008114200

21 January 2018

Identifiers

DOI

10.1074/JBC.M008114200

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2 July 2017

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