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The Pyruvate Dehydrogenase Multienzyme Complex
Angewandte Chemie International Edition in English, 1975AbstractThe three enzymes pyruvate dehydrogenase, dihydrolipoamide transacetylase, and dihydrolipoamide dehydrogenase constitute the pyruvate dehydrogenase multienzyme complex of E. coli; in mammals the complex also contains a kinase and a phosphatase.
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Disorders of pyruvate carboxylase and the pyruvate dehydrogenase complex
Journal of Inherited Metabolic Disease, 1996SummaryThe most common defect associated with deficiency of the pyruvate dehydrogenase (PDH) complex occurs in the E1 component, specifically due to mutations in the X‐linked E1α gene. Clinical sequelae of these mutations, which range from severe neonatal lactic acidosis to carbohydrate‐sensitive ataxia, can be different in males and females depending ...
B H, Robinson +3 more
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Pyruvate Dehydrogenase Complex Deficiency
2006Abstract Identical twins with the fictitious names Ann and Elizabeth were born 6 weeks prematurely to a healthy 26-year-old mother whose pregnancy was uneventful. The family history was noteworthy for epilepsy in a paternal aunt, congenital deafness in another paternal aunt, and possible mental retardation in a maternal cousin. The twins’
Peter W Stacpoole, Lesa R Gilbert
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Plant pyruvate dehydrogenase complexes
1996While there is considerable overall metabolic similarity between plant and animal cells, drastically different anatomy, physiology, and organismal requirements have led to increasing diversity between these two classes of eukaryotes. Analyses of the pyruvate dehydrogenase complex (PDC) in plant cells serve to illustrate both the similarities inherent ...
M. H. Luethy +3 more
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The pyruvate dehydrogenase multienzyme complex
Current Opinion in Structural Biology, 1992Abstract During the review period, several structures of component enzymes and domains of enzymes of this multienzyme complex were determined. Three structures of the flavoprotein component, dihydrolipoamide dehydrogenase, became available. The structure of the core component, dihydrolipoyl acetyltransferase, can in principle be constructed from the ...
Andrea Mattevi +2 more
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Pyruvate Dehydrogenase Complex Deficiencies
2012Pyruvate dehydrogenase complex (PDC) deficiencies, affecting a critical step in carbohydrate oxidative metabolism, have heterogeneous genetic causes and outcomes. There are few correlations between numerous mutations, affecting at least 10 genes, measured PDC enzyme activity, and the clinical outcome. The presentation varies by age and sex.
Suzanne D. DeBrosse, Douglas S. Kerr
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Assays of Pyruvate Dehydrogenase Complex and Pyruvate Carboxylase Activity
2011Pyruvate dehydrogenase complex (PDC) and pyruvate carboxylase (PC) are mitochondrial enzymes that provide the initial steps of the two main alternatives for pyruvate metabolism: oxidative decarboxylation vs. anaplerotic carboxylation, gluconeogenesis, and glycerogenesis.
Douglas, Kerr +2 more
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Pyruvate Dehydrogenase Complex
1969Publisher Summary This chapter discusses the multienzyme pyruvate dehydrogenase complexes, with emphasis on their structure, function, and regulation. These complexes have been isolated from Escherichia coli and from animal tissues as functional units with molecular weights in the millions.
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Regulation of the Pyruvate Dehydrogenase Multienzyme Complex
Annual Review of Nutrition, 1993To maximize catalytic efficiency in metabolic pathways of both prokaryotic and eUkaryotic cells, enzymatic components are occasionally clustered or complexed physically. Organization of multiple catalytic functions into a single enzyme complex can be accomplished in two ways.
R H, Behal +3 more
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The hormonal regulation of pyruvate dehydrogenase complex
Advances in Enzyme Regulation, 1996The pyruvate dehydrogenase complex has a central role in the regulation of mammalian metabolism as it represents the point-of-no-return in the utilization of carbohydrate. This article summarizes our studies into how signalling systems initiated by hormones binding to cell surface receptors can reach the pyruvate dehydrogenase system which is located ...
Denton, RM +6 more
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