Respiratory chain supercomplexes set the threshold for respiration defects in human mtDNA mutant cybrids (Q40272913)

19 September 2017

Respiratory chain supercomplexes set the threshold for respiration defects in human mtDNA mutant cybrids (English)

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19 September 2017

Marilena D'Aurelio

1

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19 September 2017

Carl D Gajewski

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2

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19 September 2017

Giorgio Lenaz

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3

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19 September 2017

Giovanni Manfredi

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4

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19 September 2017

1 June 2006

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19 September 2017

Human Molecular Genetics

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19 September 2017

15

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19 September 2017

13

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19 September 2017

2157-2169

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Biogenesis of mitochondria

19 September 2017

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https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Fluidity in mitochondrial membranes: thermotropic lateral translational motion of intramembrane particles

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

The random collision model and a critical assessment of diffusion and collision in mitochondrial electron transport

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Supercomplexes in the respiratory chains of yeast and mammalian mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Respiratory chain supercomplexes of mitochondria and bacteria

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Significance of respirasomes for the assembly/stability of human respiratory chain complex I.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Architecture of active mammalian respiratory chain supercomplexes.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

The mitochondrial respiratory chain is partially organized in a supercomplex assembly: kinetic evidence using flux control analysis

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Emerging functions of mammalian mitochondrial fusion and fission

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Transcomplementation between different types of respiration-deficient mitochondria with different pathogenic mutant mitochondrial DNAs

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

In vivo regulation of oxidative phosphorylation in cells harboring a stop-codon mutation in mitochondrial DNA-encoded cytochrome c oxidase subunit I.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Human cells lacking mtDNA: repopulation with exogenous mitochondria by complementation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Suppression of a mitochondrial tRNA gene mutation phenotype associated with changes in the nuclear background

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Nuclear suppression of mitochondrial defects in cells without the ND6 subunit

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Differences in assembly or stability of complex I and other mitochondrial OXPHOS complexes in inherited complex I deficiency

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Inter-mitochondrial complementation: Mitochondria-specific system preventing mice from expression of disease phenotypes by mutant mtDNA.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Very rare complementation between mitochondria carrying different mitochondrial DNA mutations points to intrinsic genetic autonomy of the organelles in cultured human cells.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Mitochondrial fusion in human cells is efficient, requires the inner membrane potential, and is mediated by mitofusins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

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/10.1093%2FHMG%2FDDL141

Assembly of Respiratory Complexes I, III, and IV into NADH Oxidase Supercomplex Stabilizes Complex I in Paracoccus denitrificans

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Respiratory complex III is required to maintain complex I in mammalian mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

A mitochondrial cytochrome b mutation causing severe respiratory chain enzyme deficiency in humans and yeast

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Cytochrome c oxidase deficiency

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Biogenesis of cytochrome oxidase-sophisticated assembly lines in the mitochondrial inner membrane

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

A possible site of superoxide generation in the complex I segment of rat heart mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Assessment of mitochondrial oxidative phosphorylation in patient muscle biopsies, lymphoblasts, and transmitochondrial cell lines

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Steady-state kinetics of ubiquinol-cytochrome c reductase in bovine heart submitochondrial particles: diffusional effects

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

Blue Native electrophoresis to study mitochondrial and other protein complexes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

High throughput two-dimensional blue-native electrophoresis: a tool for functional proteomics of mitochondria and signaling complexes

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

A Method for the Localization of Sites for Oxidative Phosphorylation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDL141

21 January 2018

Identifiers

DOI

10.1093/HMG/DDL141

1 reference

19 September 2017

1 reference