(Q46715013)

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Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects.

scientific article published on 19 September 2005

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7 January 2020

Differential inhibitory effect of long-chain acyl-CoA esters on succinate and glutamate transport into rat liver mitochondria and its possible implications for long-chain fatty acid oxidation defects (English)

1 reference

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7 January 2020

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Fátima V Ventura

1

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7 January 2020

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J Ruiter

2

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7 January 2020

L Ijlst

3

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7 January 2020

I Tavares de Almeida

4

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7 January 2020

R J A Wanders

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19 September 2005

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7 January 2020

Molecular Genetics and Metabolism

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7 January 2020

86

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7 January 2020

3

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7 January 2020

344-352

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7 January 2020

Control of mitochondrial beta-oxidation flux

1 reference

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7 January 2021

Disorders of mitochondrial fatty acyl-CoA beta-oxidation

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Clinical diagnosis of long-chain acyl-coenzyme A-dehydrogenase deficiency: use of stress and fat-loading tests.

1 reference

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Identification of very-long-chain acyl-CoA dehydrogenase deficiency in three patients previously diagnosed with long-chain acyl-CoA dehydrogenase deficiency

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Very long chain acyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid oxidation in fibroblasts

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Disorders of mitochondrial beta-oxidation: prenatal and early postnatal diagnosis and their relevance to Reye's syndrome and sudden infant death

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Combined enzyme defect of mitochondrial fatty acid oxidation

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Disorders of mitochondrial long-chain fatty acid oxidation

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Molecular and physiological aspects of adenine nucleotide trasport in mitochondria

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Application of modular control analysis to inhibition of the adenine nucleotide translocator by palmitoyl-CoA.

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Peripheral sensory-motor polyneuropathy, pigmentary retinopathy, and fatal cardiomyopathy in long-chain 3-hydroxy-acyl-CoA dehydrogenase deficiency

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Fatty acid oxidation disorders: A new class of metabolic diseases

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Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency--diagnosis, plasma carnitine fractions and management in a further patient

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Pathology of skeletal muscle and impaired respiratory chain function in long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency with the G1528C mutation

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Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency with the G1528C mutation: clinical presentation of thirteen patients.

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Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: variable expressivity of maternal illness during pregnancy and unusual presentation with infantile cholestasis and hypocalcaemia

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A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women

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Trifunctional protein deficiency: three families with significant maternal hepatic dysfunction in pregnancy not associated with E474Q mutation

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Molecular and phenotypic heterogeneity in mitochondrial trifunctional protein deficiency due to ?-subunit mutations

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Mitochondrial trifunctional protein deficiency: a severe fatty acid oxidation disorder with cardiac and neurologic involvement

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A case of mitochondrial trifunctional protein deficiency diagnosed by acylcarnitine profile and DNA analysis in a dried blood spot of a 4-day-old boy.

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Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid β-oxidation disorders

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Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acidβ-oxidation intermediates on mitochondrial oxidative phosphorylation

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On the mechanism of malonyl-CoA-independent fatty acid synthesis. I. The mechanism of elongation of long-chain fatty acids by acetyl-CoA

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Measurement of protein using bicinchoninic acid

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Studies on mitochondrial oxidative phosphorylation in permeabilized human skin fibroblasts: application to mitochondrial encephalomyopathies

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Assay of citric acid cycle intermediates and related compounds--update with tissue metabolite levels and intracellular distribution

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Metabolite Transport in Mitochondria

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An appraisal of the functional significance of the inhibitory effect of long chain acyl-CoAs on mitochondrial transports

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Palmitoyl-CoA inhibits the mitochondrial inner membrane anion-conducting channel

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Organization and sequence of the gene for the human mitochondrial dicarboxylate carrier: evolution of the carrier family

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Lactic acidosis in long-chain fatty acid beta-oxidation disorders

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