E 1 (EC 1.2. 4.4) uses thiamine pyrophosphate (TPP) as a catalytic cofactor. E 1 catalyzes both the decarboxylation of the α-ketoacid and the subsequent reductive acylation of the lipoyl moiety (another catalytic cofactor) that is covalently bound to E 2 .
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The mammalian mitochondrial branched-chain α-keto acid dehydrogenase (BCKD) complex catalyzes the rate-limiting step in the catabolism of branched-chain amino ...
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The decarboxylase (E1) component of the mammalian branched-chain-keto α-acid dehydrogenase (BCKD) complex is a thiamine pyrophosphate (TPP)-dependent enzyme ...
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The branched-chain alpha-keto acid dehydrogenase complex is a combination of enzymes responsible for the degradation of the branched chain amino ...
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Branched-chain alpha-keto acid dehydrogenase complex is the rate-limiting enzyme in the catabolism of branched-chain amino acids in skeletal muscle. Termes manquants : cofactor | Doit inclure : cofactor
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Although deficiency of the branched-chain ketoacid dehydrogenase (BCKDC) and associated elevations in the BCAAs and their ketoacids have been recognized as the ...
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The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It contains multiple copies of ...
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29 nov. 2005 · Component enzyme of branched-chain α-keto acid dehydrogenase complex : lipoamide acyltransferase component ... Summary: The bfmBAA and bfmBAB ...
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The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO2. It is a multienzyme complex ...
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E1, which contains a thiamine diphosphate cofactor, catalyzes the binding of the 2-oxo acid to the lipoyl group of E2, which then transfers an acyl group (the ...
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27 févr. 2007 · 2The abbreviations used are: ThDP, thiamine diphosphate; PDC, pyruvate dehydrogenase complex; BCKDC, branched-chain α-keto acid ...
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Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.
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The BCKDHA gene encodes the E1-alpha subunit of the branched-chain alpha-keto acid (BCAA) dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial ...
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Branched-chain alpha-keto acid dehydrogenase (BCKDH) complexes of lymphoblastoid cell lines derived from patients with classical maple syrup urine disease ...
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Cross-Talk between Thiamin Diphosphate Binding and Phosphorylation Loop Conformation in Human Branched-Chain Alpha-Keto Acid Decarboxylase/Dehydrogenase.
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