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

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Shorter term and longer term regulation of pyruvate dehydrogenase kinases,The reaction connects glycolysis to oxidative metabolism, and to FFA synthesis, resulting in the irretrievable loss of glucose carbon (animals lack the means of converting acetylCoA to glucose). Some cells in the central nervous system are critically dependent upon glucose oxidation for survival and
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,Regulation of branched-chain α-keto acid dehydrogenase complex in rat liver and skeletal muscle by g step in the catabolism of branched-chain amino acids. The enzyme complex is subject to covalent modification; a phosphorylation / dephosphorylation cycle regulates the enzyme activity. It has been reported that dietary protein has a great effect on the activity state of the enzyme complex in rat l
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Long-term regulation and promoter analysis of mammalian pyruvate dehydrogenase complex,ds to form acetyl-CoA in the mitochondria. Acetyl-CoA is then utilized for either energy production by the tricarboxylic acid cycle or energy storage by the lipogenic pathway. This enzyme complex contains multiple copies of three catalytic components: pyruvate dehydrogenase (E1), dihydrolipoamide ac
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Human defects of the pyruvate dehydrogenase complex,in the intrauterine environment where energy production depends primarily on glucose oxidation. The now well-established occurrence of these defects presents a challenge to provide physiological explanations of what factors determine survival and adaptation of affected individuals. Unfortunately, fo
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Multigenic basis for maple syrup urine disease with emphasis on mutations in branched chain dihydroto “run in families”(Garrod, 1908, 1923). By the 1950s, this understanding extended to knowing specific enzyme defects for several inborn errors. Thus, the stage was set for describing new disorders of which maple syrup urine disease (MSUD) was one. MSUD was first described clinically in 1954 by Men
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