domingo, 7 de septiembre de 2014

Branched-chain alpha-keto acid dehydrogenase complex

The branched-chain α-ketoacid dehydrogenase complex (BCKDC) is a multi-subunit complex of enzymes that is found on the mitochondrial inner membrane. This enzyme complex catalyzes the oxidative decarboxylation of branched, short-chain alpha-ketoacids. BCKDC is a member of the mitochondrial α-ketoacid dehydrogenase complex family comprising pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, key enzymes that function in the Krebs cycle.
Cofactors
This complex requires the following 5 cofactors:

Thiamine pyrophosphate
FAD
NAD+
Lipoate
Coenzyme A

Biological Function
In animal tissue, BCKDC catalyzes an irreversible step in the catabolism of branched-chain amino acids—namely L-isoleucine, L-valine, and L-leucine and their derivatives (L-alpha-keto-beta-methylvalerate, alpha-ketoisovalerate, and alpha-ketoisocaproate, respectively). In bacteria, this enzyme participates...

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