Pyruvate dehydrogenase deficiencies

Blass, John P.

doi:10.1007/978-94-009-9215-3_15

Cited by 12 publications

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“…A number of congenital neurological disorders have been associated with abberations of pyruvate metabolism (for review see Blass, 1981; Sorbi and Blass, 1982 a ). Impairment of the catalytic capacity, including abnormal coenzyme binding, has been demonstrated in some patients (Blass, 1980; Sheu et al, 1981). Abnormality of the phosphorylation regulatory system has also been suggested (Robinson and Sherwood, 1975; De Vivo et al, 1979; Sorbi and Blass, 1982 b ).…”

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Pyruvate Dehydrogenase Phosphate (PDH_b) Phosphatase in Brain: Activity, Properties, and Subcellular Localization

Sheu

Lai

Blass

1983

Journal of Neurochemistry

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The activity of pyruvate dehydrogenase phosphate (PDHb) phosphatase in rat brain mitochondria and homogenate was determined by measuring the rate of activation of purified, phosphorylated (i.e., inactive) pyruvate dehydrogenase complex (PDHC), which had been purified from bovine kidney and inactivated by phosphorylation with Mg . ATP. The PDHb phosphatase activity in purified mitochondria showed saturable kinetics with respect to its substrate, the phospho-PDHC. It had a pH optimum between 7.0 and 7.4, depended on Mg and Ca, and was inhibited by NaF and K-phosphate. These properties are consistent with those of the highly purified enzyme from beef heart. On subcellular fractionation, PDHb phosphatase copurified with mitochondrial marker enzymes (fumarase and PDHC) and separated from a cytosolic marker enzyme (lactate dehydrogenase) and a membrane marker enzyme (acetylcholinesterase), suggesting that it, like its substrate, is located in mitochondria. PDHb phosphatase had similar kinetic properties in purified mitochondria and in homogenate: dependence on Mg and Ca, independence of dichloroacetate, and inhibition by NaF and K-phosphate. These results are consistent with there being only one type of PDHb phosphatase in rat brain preparations. They support the validity of the measurements of the activity of this enzyme in brain homogenates.

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Pyruvate Dehydrogenase Phosphate (PDH_b) Phosphatase in Brain: Activity, Properties, and Subcellular Localization

Sheu

Lai

Blass

1983

Journal of Neurochemistry

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Problems in the Congenital Lactic Acidoses

Leonard

1982

Ciba Foundation Symposium 87 ‐ Metabolic Acidosis

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Diseases of Skeletal Muscle

Edwards

Jones

1983

Comprehensive Physiology

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The sections in this article are: History Clinical Presentation of Muscle Disease Weakness Fatigue Myoglobinuria Unusual Function Investigation of Patients with Muscle Disease Clinical History and Examination Blood Biochemistry Electromyography Muscle Biopsy Nuclear Magnetic Resonance Measurement of Muscle Cross‐Sectional Area Tests of Muscle Function Isolated Human Muscle Preparations Exercise and Fasting as Provocation Tests Sudies of Protein Turnover Analysis of Muscle Weakness Dystrophies Inflammatory Myopathies Atrophy and Hypertrophy Factors Affecting Protein Turnover in Muscle Neuropathies Analysis of Abnormal Fatigability Muscle Fatigue in Normal Subjects Myasthenia Gravis Disorders of Energy Metabolism Disorders of Mitochondrial Oxidative Metabolism Disorders of Fat Metabolism Changes in Muscle Function Disordered Function Myotonic Syndromes Periodic Paralysis Malignant Hyperpyrexia Muscle Pain, Cramps, and Contractures Drug‐Induced Myopathies Mechanisms Underlying Muscle Disease Vascular and Humoral Influences Influence of Nerve on Muscle Disorders of the Cell Membrane Muscle Protease Activity Inflammatory Disorders of Muscle Overuse and Disuse as Causes of Muscle Damage Prospects For Treatment of Muscle Disease

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Pyruvate dehydrogenase deficiencies

Cited by 12 publications

References 66 publications

Pyruvate Dehydrogenase Phosphate (PDH_b) Phosphatase in Brain: Activity, Properties, and Subcellular Localization

Pyruvate Dehydrogenase Phosphate (PDH_b) Phosphatase in Brain: Activity, Properties, and Subcellular Localization

Problems in the Congenital Lactic Acidoses

Diseases of Skeletal Muscle

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Pyruvate dehydrogenase deficiencies

Cited by 12 publications

References 66 publications

Pyruvate Dehydrogenase Phosphate (PDHb) Phosphatase in Brain: Activity, Properties, and Subcellular Localization

Pyruvate Dehydrogenase Phosphate (PDHb) Phosphatase in Brain: Activity, Properties, and Subcellular Localization

Problems in the Congenital Lactic Acidoses

Diseases of Skeletal Muscle

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Product

Resources

About

Pyruvate Dehydrogenase Phosphate (PDH_b) Phosphatase in Brain: Activity, Properties, and Subcellular Localization

Pyruvate Dehydrogenase Phosphate (PDH_b) Phosphatase in Brain: Activity, Properties, and Subcellular Localization