Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria (Q34240411)

31 July 2017

title

Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria (English)

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31 July 2017

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Watmough NJ

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31 July 2017

Bindoff LA

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31 July 2017

Birch-Machin MA

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31 July 2017

Jackson S

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31 July 2017

Bartlett K

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31 July 2017

Ragan CI

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31 July 2017

Poulton J

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31 July 2017

Gardiner RM

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31 July 2017

Sherratt HS

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31 July 2017

Turnbull DM

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31 July 2017

language of work or name

English

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publication date

1 January 1990

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Journal of Clinical Investigation

31 July 2017

published in

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31 July 2017

volume

85

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31 July 2017

issue

1

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31 July 2017

page(s)

177-184

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Metabolism of 2-hexadecynoate and inhibition of fatty acid elongation

31 July 2017

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The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria

28 July 2018

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Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis

28 July 2018

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Quantitation of rabbit cytochrome P-450, form 2, in microsomal preparations bound directly to nitrocellulose paper using a modified peroxidase-immunostaining procedure

28 July 2018

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How mitochondria import proteins

28 July 2018

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28 July 2018

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Tetracyano-2,2-bipyridineiron(iii), an improved electron acceptor for the spectrophotometric assay of beta-oxidation and of succinate dehydrogenase in intact mitochondria

28 July 2018

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28 July 2018

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Factors controlling the rate of fatty acid -oxidation in rat liver mitochondria

28 July 2018

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28 July 2018

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Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase

28 July 2018

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28 July 2018

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

28 July 2018

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28 July 2018

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28 July 2018

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Skeletal muscle mitochondrial beta-oxidation. A study of the products of oxidation of [U-14C]hexadecanoate by h.p.l.c. using continuous on-line radiochemical detection

28 July 2018

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28 July 2018

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Deficiency of subunits of Complex I and mitochondrial encephalomyopathy

28 July 2018

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Deficiency of the reduced nicotinamide adenine dinucleotide dehydrogenase component of complex I of mitochondrial electron transport. Fatal infantile lactic acidosis and hypermetabolism with skeletal-cardiac myopathy and encephalopathy

28 July 2018

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Congenital deficiency of two polypeptide subunits of the iron-protein fragment of mitochondrial complex I.

28 July 2018

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28 July 2018

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Respiratory chain defects in the mitochondria of cultured skin fibroblasts from three patients with lacticacidemia

28 July 2018

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28 July 2018

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Restriction enzyme analysis of the mitochondrial genome in mitochondrial myopathy

28 July 2018

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The mitochondrial electron transport and oxidative phosphorylation system

28 July 2018

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Measurement of the acyl-CoA intermediates of beta-oxidation by h.p.l.c. with on-line radiochemical and photodiode-array detection. Application to the study of [U-14C]hexadecanoate oxidation by intact rat liver mitochondria

28 July 2018

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Mitochondrial heat-shock protein hsp60 is essential for assembly of proteins imported into yeast mitochondria

28 July 2018

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Duplications of mitochondrial DNA in mitochondrial myopathy

28 July 2018

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Detection of specific sequences among DNA fragments separated by gel electrophoresis

28 July 2018

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28 July 2018

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Short-chain acyl-CoA dehydrogenase deficiency associated with a lipid-storage myopathy and secondary carnitine deficiency.

28 July 2018

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The absorbance coefficient of beef heart cytochrome c1

28 October 2018

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Myopathy associated with abnormal lipid metabolism in skeletal muscle

28 October 2018

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Mitochondrial myopathies: disorders of the respiratory chain and oxidative phosphorylation

28 October 2018

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12 December 2020

Characterization of an RNA polymerase activity from HeLa cell mitochondria, which initiates transcription at the heavy strand rRNA promoter and the light strand promoter in human mitochondrial DNA

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12 December 2020

Mitochondrial myopathies: defects in beta-oxidation

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12 December 2020

A method for the preparation of 3-ketoacyl-CoA derivatives

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12 December 2020

Analysis of enzymatically amplified β-globin and HLA-DQα DNA with allele-specific oligonucleotide probes

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Mitochondrial encephalomyopathy. Association with an NADH dehydrogenase deficiency

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12 December 2020

Mitochondrial myopathies

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12 December 2020

Molecular basis of mitochondrial myopathies: polypeptide analysis in complex-I deficiency

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12 December 2020

MTTOCHONDRIAL DNA DELETION IN ENCEPHALOMYOPATHY

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12 December 2020

Fatal lactic acidosis in infancy with a defect of complex III of the respiratory chain

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12 December 2020

Effect of temperature on the spectral properties of some ferrocytochromes

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12 December 2020

Inhibition of general acyl CoA dehydrogenase by electron transfer flavoprotein semiquinone

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Identifiers

DOI

10.1172/JCI114409

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31 July 2017

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31 July 2017

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