BCKD Complex (50 μg)

Source: Bovine kidney

Purity: > 90% by SDS-PAGE, subunit apparent Mr's 52,000 (E2), 46,000 (E1a), 35,000 (E1β). Apparent Mr ~ 3,200,000 by sedimentation equilibrium centrifugation.

Supplied: 50 μg in 50 μl 50 mM Tris-HCl pH 7.3 buffer containing 14 mM β-mercaptoethanol, 1 mM benzamidine, 0.1 mM phenylmethanesulfonyl fluoride, 1 mM EDTA and 10% glycerol.

Activity: ~ 8-10 μmol of NADH formed per min per mg of protein with a-ketoisovalerate as substrate. Maintain in aliquots at -70° C. Avoid repeated thawing.

Synonyms: Branched-Chain a-Keto Acid Dehydrogenase Complex; BCKDC; BCKD Complex; BCKDH Complex

Background: The branched-chain a-keto acid dehydrogenase complex (BCKDC) catalyzes the oxidative decarboxylation of the branched-chain a-keto acids derived from the essential amino acids, leucine, isoleucine and valine. It is a mitochondrial multi-enzyme complex that is regulated by reversible phosphorylation. Phosphorylation of the decarboxylase component of the complex (E1) by BCKDC kinase prevents the catabolism of the a-keto acids. Dephosphorylation by mitochondrial BCKDC-phosphatase reactivates the complex. Unlike the phosphatase, BCKDC kinase is tightly associated with the complex. The kinase is regulated by allosteric effectors as well as factors that affect its level of expression. It is inhibited potently by the a-keto acid produced by leucine transamination, and by starvation for dietary protein, which causes increased expression of the enzyme. Additionally, thyroid hormone induces expression of the kinase. In contrast, glucocorticoids and peroxisome proliferator-activated receptor ligands repress expression of the kinase.


Related ProductsBCKDC (100 μg) ♦ BCKD Kinase Kit ♦ PDC (10 μg) ♦ PDC (50 μg)

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