Titlebook: Alpha-Keto Acid Dehydrogenase Complexes; Mulchand S. Patel,Thomas E. Roche,Robert A. Harris Book 1996 Birkh?user Verlag 1996 Molekularbiol

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Lipoamide dehydrogenase, 2-electron reduced enzyme. Mutagenesis studies and the use of a modified flavin cofactor revealed the various factors involved in this stabilization in which also the core component of the complex has a function.

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Shorter term and longer term regulation of pyruvate dehydrogenase kinases, conservation of glucose by substitution of lipid fuels in cells able to oxidise them is of vital importance during carbohydrate deprivation. Regulation of the PDH complex is therefore of crucial importance in glucose homeostasis.

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,Molecular defects of the branched-chain α-keto acid dehydrogenase complex: Maple syrup urine diseasulting branched-chain acyl-coenzyme A (CoA) to enoyl CoA (Tanaka and Rosenberg, 1983). The oxidative decarboxylation of the BCKAs is performed by the branched-chain α-keto acid dehydrogenase complex (BCKDH), a constituent of all mammalian tissues which is associated with the mitochondrial inner membrane.

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Hormonal and nutritional modulation of PDHC activity status,acteristics of the mitochondrial pyruvate dehydrogenase holocomplex (PDHC), which catalyses the oxidative decarboxylation of pyruvate to acetyl CoA. This chapter will review the regulation of PDHC by hormones and nutrients within the context of the competition between fatty acids (FA) and glucose as oxidative substrates.

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