Results 211 to 220 of about 94,657 (246)

Pyruvate Dehydrogenase Complex Deficiency

2016
Pyruvate dehydrogenase complex (PDHc) deficiency usually first manifests at a young age and is rarely diagnosed in adulthood. The clinical picture varies from neonatal death with overwhelming lactic acidosis to a relatively benign course early in life.
Suzanne D. DeBrosse, Douglas S. Kerr
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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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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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Effect of mycoplasma infection on pyruvate dehydrogenase complex activity of normal and pyruvate dehydrogenase complex-deficient fibroblasts

Experimental Cell Research, 1984
The fermentative mycoplasmas A. laidlawii JS, M. hyorhinis DBS-50, M. hyorhinis GDL and M. pneumoniae FH have very high apparent activities of pyruvate dehydrogenase (PDH) (EC 1.2.4.1) and pyruvate dehydrogenase complex (PDHC). Infection of normal and PDHC-deficient fibroblasts with these mycoplasma species resulted in a marked increase of the specific
G J, McGarrity, G, Constantopoulos, J A, Barranger   +2 more
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Specificity of the pyruvate dehydrogenase kinase for pyruvate dehydrogenase component bound to the surface of the kidney pyruvate dehydrogenase complex and evidence for intracore migration of pyruvate dehydrogenase component

Biochemistry, 1983
Using the bovine kidney pyruvate dehydrogenase complex we have investigated the mechanism whereby about three pyruvate dehydrogenase (active form) kinase molecules, tightly bound to the dihydrolipoyl transacetylase core, can rapidly phosphorylate and inactivate about 20 pyruvate dehydrogenase (active form) (PDHa) tetramers which are also bound to the ...
D R, Brandt, T E, Roche
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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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Characterization of the inhibition of Escherichia coli pyruvate dehydrogenase complex by pyruvate

Biochemical and Biophysical Research Communications, 1991
The E. coli pyruvate dehydrogenase complex was inhibited by pyruvate in absence of its cofactor, NAD+. The inhibition was found to increase with pH and phosphate concentration of the buffer and decrease with its ionic strength. The inhibition profile was different with MOPS buffer. No radioactivity was found in the enzyme, when the latter was incubated
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Regulation of the activity of the pyruvate dehydrogenase complex

Advances in Enzyme Regulation, 2002
Robert A, Harris, Melissa M, Bowker-Kinley, Boli, Huang, Pengfei, Wu   +3 more
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MOBILITY OF THE PYRUVATE DEHYDROGENASE COMPONENT IN THE KIDNEY PYRUVATE DEHYDROGENASE COMPLEX

Annals of the New York Academy of Sciences, 1982
Thomas E. Roche, Douglas R. Brandt, Mary L. Pratt   +2 more
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