pyruvate dehydrogenase
Central Hypoventilation Syndrome in Pyruvate Dehydrogenase Complex Deficiency
... 74:1034-1040; central hypoventilation syndrome, doxap- ram, almitrine, lactic acidosis, pyruvate dehydrogenase complex.. In recent years, more than 50 patients have been reported with va[r] ...
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Defects in the E2 lipoyl transacetylase and the X lipoyl containing component of the pyruvate dehydrogenase complex in patients with lactic acidemia
... blotting with anti-pyruvate dehydrogenase complex antiserum which were not located in the E1 (alpha and beta) component of the complex. One of these patients had an enzymatically demonstrable deficiency in ...
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Metabolic plasticity maintains proliferation in pyruvate dehydrogenase deficient cells
... The pyruvate dehydrogenase complex (PDH) occupies a crucial node in glucose metabolism, as it oxidatively decarboxylates pyruvate generated from glycolysis or other pathways to generate acetyl-CoA ...
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Pyruvate Dehydrogenase Complex Activity in Normal and Deficient Fibroblasts
... purified pyruvate dehydrogenase phosphatase and high concentrations of Mg ++ and Ca ++ , or in some cases by adding the metal ions alone to the cell homogenate ...
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Engineering Acetyl Coenzyme A Supply: Functional Expression of a Bacterial Pyruvate Dehydrogenase Complex in the Cytosol of Saccharomyces cerevisiae
... yeast pyruvate dehydrogenase complex Pdb1 (pyruvate dehydrogenase beta-subunit [E1  ]), Lat1 (dihydrolipoamide acetyltransferase [E2]), and Pdx1 (pyruvate dehydrogenase com- ...
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The pivotal role of pyruvate dehydrogenase kinases in metabolic flexibility
... the pyruvate dehydrogenase complex ...by pyruvate dehydrogenase (PDH) kinase (PDK) is crucial to maintain energy homeostasis under some extreme nutritional conditions in ...
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Induction and Activities of Pyruvate Dehydrogenase and -ketoglutarate Dehydrogenase in Type 2 Diabetic Patients and Therapy with Vitamin B1
... The method used for the PDE1 assay was the same reported previously [29]. However, in order to adapt this method for the microplate reader the reaction mixture volumes were reduced as mentioned below. This protocol ...
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Rearrangement of mitochondrial pyruvate dehydrogenase subunit dihydrolipoamide dehydrogenase protein–protein interactions by the MDM2 ligand nutlin-3
... Based on the information introduced above, cell-based quantitative proteomics screens were set up that aimed to identify dominant subcellular organelles that are affected rel- atively early after Nutlin-3 treatment and ...
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Pyruvate Dehydrogenase Complex Deficiency as a Cause of Subacute Necrotizing Encephalopathy (Leigh Disease)
... These findings, considered together with the aggregate data in the liter- ature, suggest strongly that pyruvate dehydrogenase corn- plex deficiency is the basic defect in a subgroup of p[r] ...
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Deficiency of the pyruvate dehydrogenase component in pyruvate dehydrogenase complex deficient human fibroblasts Immunological identification
... of pyruvate dehydrogenase complex were analyzed using an immunoblot technique and specific ...the pyruvate dehydrogenase complex activity was due to a reduction in the amount of the E1 ...
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Pyruvate dehydrogenase complex stimulation promotes immunometabolic homeostasis and sepsis survival
... activate Pyruvate dehydrogenase kinase 1 (PDK1) (7), which inhibits the pyruvate dehydroge- nase complex (PDC) by reversible phosphorylation of any 1 of 3 serine residues on its E1α subunit ...
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Effects of insulin infusion on human skeletal muscle pyruvate dehydrogenase, phosphofructokinase, and glycogen synthase Evidence for their role in oxidative and nonoxidative glucose metabolism
... or pyruvate dehydrogenase (PDH) in skeletal muscle regulates intracellular glucose metabolism, subjects were studied basally and during euglycemic insulin infusions of 12, 30, and 240 mU/m2 X ...
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Carbohydrate Metabolism in the Toxoplasma gondii Apicoplast: Localization of Three Glycolytic Isoenzymes, the Single Pyruvate Dehydrogenase Complex, and a Plastid Phosphate Translocator
... to pyruvate kinase, are present in the ...and pyruvate kinase were found to localize to the ...single pyruvate dehydrogenase complex, which was located in the apicoplast and absent in the ...
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Specific inhibition by synthetic analogs of pyruvate reveals that the pyruvate dehydrogenase reaction is essential for metabolism and viability of glioblastoma cells
... excess pyruvate fails to restore the activity lost during preincubation with AcPH (Figures 2B, ...when pyruvate is present during phosphonate binding is PDHC protected from inactivation by AcPH ...with ...
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Lactic Acidosis in Three Sibs Due to Defects in Both Pyruvate Dehydrogenase and α-Ketoglutarate Dehydrogenase Complexes
... They had chronic metabolic acidosis associated with elevated blood levels of lactate, pruvate, and alanine.. Two of the children excreted large amounts of pyruvic and a-ketoglutaric acid[r] ...
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Ketonic Diet in the Management of Pyruvate Dehydrogenase Deficiency
... me, $-hydroxybutyrate, and citrate and urinary lactate, pyruvate, alanine, and /9-hydroxybutyrate were measured after the patients had eaten the “¿normal― or high-fat diets for five [r] ...
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Engineering of Serine-Deamination pathway, Entner-Doudoroff pathway and pyruvate dehydrogenase complex to improve poly(3-hydroxybutyrate) production in Escherichia coli
... There has been a range of studies to evaluate PHB pro- duction in E. coli. Enhancing the availability of the precur- sor acetyl-CoA or/and cofactor NADPH increased the production of PHB. By overexpressing the fructose- ...
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Ketonic Diet in the Management of Pyruvate Dehydrogenase Deficiency
... PDH deficiency developed severe lactic acidosis after a short period of increased carbohydrate intake although he had minimal lactate acidemia on a regular diet.@ A second patient had pe[r] ...
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(HR=1.81, 95% CI: 1.42-2.31, P<0.001 ). Moreover, PDK1 was related to T grade (OR=1.72, 95% CI: 1.27-2.31, P<0.001 Conclusion: The present meta-analysis showed that high PDK1 expression predicts poor prognosis and T
... that pyruvate dehydrogenase kinase 1 (PDK1) is dysregu- lated in various tumors and that PDK1 overexpression is associated with survival and clinicopathological features in patients with ...
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Tribute to P L Lutz: putting life on `pause' – molecular regulation of hypometabolism
... For example, genes that are upregulated during hibernation in ground squirrels include ␣2-macroglobulin in liver, moesin in intestine, isozyme 4 of pyruvate dehydrogenase kinase PDK4 and[r] ...
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