Results 291 to 300 of about 158,743 (322)

Complexities of the pyruvate dehydrogenase complex

Neurology, 1998
If one were to throw a dart at the center of a biochemical chart for intermediary metabolism, it would land on acetyl-CoA. This metabolite is at the convergence of pyruvate, fatty acid, and ketone body metabolism. Condensation of acetyl-CoA with oxaloacetate forms citric acid, the entry point into the tricarboxylic acid cycle.
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Disorders of the pyruvate dehydrogenase complex

Journal of Inherited Metabolic Disease, 1986
AbstractPyruvate dehydrogenase deficiency may be a non‐specific consequence of many different neurological degenerative disorders. There are also serious methodological problems in estimating the activity of this enzyme complex.
D, Stansbie, S J, Wallace, C, Marsac
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Regulation of the pyruvate dehydrogenase complex

Biochemical Society Transactions, 2006
The PDC (pyruvate dehydrogenase complex) plays a central role in the maintenance of glucose homoeostasis in mammals. The carbon flux through the PDC is meticulously controlled by elaborate mechanisms involving post-translational (short-term) phosphorylation/dephosphorylation and transcriptional (long-term) controls.
M S, Patel, L G, Korotchkina
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The pyruvate dehydrogenase complex during aging

Mechanisms of Ageing and Development, 1984
Acetylcholine synthesis and pyruvate oxidation decline with age. To determine the role of the pyruvate dehydrogenase complex in these age-related deficits, its activity and activation state were measured in vivo and in vitro in the brains of 3-, 10- and 30-month-old mice.
H, Ksiezak-Reding, C, Peterson, G E, Gibson   +2 more
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The Pyruvate Dehydrogenase Multienzyme Complex

Angewandte Chemie International Edition in English, 1975
AbstractThe 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, 1996
SummaryThe 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, N, MacKay, K, Chun, M, Ling   +3 more
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Pyruvate Dehydrogenase Complex Deficiency

2006
Abstract 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

1996
While 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, J. A. Miernyk, N. R. David, D. D. Randall   +3 more
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The pyruvate dehydrogenase multienzyme complex

Current Opinion in Structural Biology, 1992
Abstract 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, Aart de Kok, Richard N. Perham   +2 more
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Pyruvate Dehydrogenase Complex Deficiencies

2012
Pyruvate 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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