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Pyruvate Dehydrogenase Complex Deficiency Disease

Known as: Intermittent Ataxia with Pyruvate Dehydrogenase Deficiency, ATAXIA, INTERMITTENT, WITH ABNORMAL PYRUVATE METABOLISM, Deficiency, PDHC 
An inherited metabolic disorder caused by deficient enzyme activity in the PYRUVATE DEHYDROGENASE COMPLEX, resulting in deficiency of acetyl CoA and… 
National Institutes of Health

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Narrower (3)

Ataxia with Lactic Acidosis, Type IPyruvate Dehydrogenase E2 DeficiencyPyruvate Dehydrogenase E3-Binding Protein Deficiency
Enzyme DeficiencyIn BloodLeigh DiseaseMicrobiological

Papers overview

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1984
1984
A re-evaluation of the role of mitochondrial pyruvate transport in the hormonal control of rat liver mitochondrial pyruvate metabolism.
The inhibitor of mitochondrial pyruvate transport alpha-cyano-beta-(1-phenylindol-3-yl)-acrylate was used to inhibit… 
Highly Cited
1983
Highly Cited
1983
The protein phosphatases involved in cellular regulation. 3. Fatty acid synthesis, cholesterol synthesis and glycolysis/gluconeogenesis.
The nature of the protein phosphatases involved in the regulation of glycolysis/gluconeogenesis, fatty acid synthesis and… 
Highly Cited
1978
Highly Cited
1978
The effect of fatty acids on the regulation of pyruvate dehydrogenase in perfused rat liver.
The effect of fatty acids on the rate of pyruvate decarboxylation was studied in perfused livers from fed rats. The production of… 
Highly Cited
1976
Highly Cited
1976
Glucose metabolism in perfused skeletal muscle. Pyruvate dehydrogenase activity in starvation, diabetes and exercise.
1. The interconversion of pyruvate dehydrogenase between its inactive phosphorylated and active dephosphorylated forms was… 
Highly Cited
1976
Highly Cited
1976
The elementary reactions of the pig heart pyruvate dehydrogenase complex. A study of the inhibition by phosphorylation.
1. A method was devised for preparing pig heart pyruvate dehydrogenase free of thiamin pyrophosphate (TPP), permitting studies of… 
Highly Cited
1969
Highly Cited
1969
Growth and Sporulation of Bacillus subtilis Mutants Blocked in the Pyruvate Dehydrogenase Complex
Two “ACE” mutants of Bacillus subtilis which require acetate for growth on glucose minimal medium have been isolated. They do not… 
1968
1968
Role of Pyruvate and S-Adenosylmethionine in Activating the Pyruvate Formate-Lyase of Escherichia coli
The pyruvate formate-lyase activity of extracts of Escherichia coli is stimulated and the dilution effect is abolished by the… 
1968
1968
Effects of epinephrine on glucose transport and metabolism in adipose tissue of normal and hypothyroid rats.
Epinephrine increases the oxidation of glucose in adipose tissue even when its lipolytic effects are markedly reduced or… 
Review
1964
Review
1964
Studies on Tyrosinosis: 1, Effect of Low-tyrosine and Low-phenylalanine Diet
not improved by operation further emboli occurred in two, and two had emboli for the first time during the follow-up years. Three… 
Highly Cited
1963
Highly Cited
1963
PYRIDOXAMINE-PYRUVATE TRANSAMINASE. II. CHARACTERISTICS OF THE ENZYME.