Mitochondrial respiratory chain dysfunction caused by coenzyme Q deficiency (Q35718352)

9 August 2017

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title

Mitochondrial respiratory chain dysfunction caused by coenzyme Q deficiency (English)

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9 August 2017

2

Arnold Munnich

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9 August 2017

3

Agnès Rötig

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9 August 2017

Pierre Rustin

1

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9 August 2017

language of work or name

English

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publication date

1 January 2004

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9 August 2017

published in

Methods in Enzymology

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9 August 2017

volume

382

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9 August 2017

page(s)

81-88

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Biochemical, physiological and medical aspects of ubiquinone function

9 August 2017

cites work

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Biochemical functions of coenzyme Q10

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Plasma ubiquinol-10 as a marker for disease: is the assay worthwhile?

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The protonmotive Q cycle: A general formulation

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Superoxide-induced massive apoptosis in cultured skin fibroblasts harboring the neurogenic ataxia retinitis pigmentosa (NARP) mutation in the ATPase-6 gene of the mitochondrial DNA.

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Antioxidant-derived prooxidant formation from ubiquinol

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Selective targeting of a redox-active ubiquinone to mitochondria within cells: antioxidant and antiapoptotic properties

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Prevention of mitochondrial oxidative damage using targeted antioxidants

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Progress on the mitochondrial permeability transition pore: regulation by complex I and ubiquinone analogs.

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Dual involvement of coenzyme Q10 in redox signaling and inhibition of death signaling in the rat heart mitochondria

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Coenzyme Q10 depletion is comparatively less detrimental to human cultured skin fibroblasts than respiratory chain complex deficiencies

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Coenzyme Q10 reverses pathological phenotype and reduces apoptosis in familial CoQ10 deficiency

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Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits

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Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy

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Mitochondrial encephalomyopathy with coenzyme Q10 deficiency

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A case of mitochondrial encephalomyopathy associated with a muscle coenzyme Q10 deficiency.

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Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency

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7 January 2021

Neonatal presentation of coenzyme Q10 deficiency

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7 January 2021

Familial cerebellar ataxia with muscle coenzyme Q10 deficiency

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7 January 2021

Coenzyme Q-responsive Leigh's encephalopathy in two sisters

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Determination of individual ubiquinone homologs by mass spectrometry and high performance liquid chromatography

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Simultaneous determination of ubiquinone-10 and ubiquinol-10 in tissues and mitochondria by high performance liquid chromatography

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Coenzyme Q10 levels in idiopathic and varicocele-associated asthenozoospermia

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10.1016/S0076-6879(04)82005-6

Identifiers

DOI

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9 August 2017

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