Determination of branched-chain α-keto acid dehydrogenase activity state and branched-chain α-keto acid dehydrogenase kinase activity and protein in mammalian …
N Nakai, R Kobayashi, KM Popov, RA Harris… - Methods in …, 2000 - Elsevier
The branched-chain α-keto acid dehydrogenase (BCKDH) complex, a large multienzyme
complex located in the mitochondrial matrix space, catalyzes oxidative decarboxylation of
branched-chain α-keto acids (α-ketoisocaproate, α-keto-β-methylvalerate, and α-
ketoisovalerate) derived from transamination of the corresponding branched-chain amino
acids (leucine, isoleucine, and valine). The reaction catalyzed by the BCKDH complex is
considered the rate-limiting step for the catabolism of all three of the branched-chain amino …
complex located in the mitochondrial matrix space, catalyzes oxidative decarboxylation of
branched-chain α-keto acids (α-ketoisocaproate, α-keto-β-methylvalerate, and α-
ketoisovalerate) derived from transamination of the corresponding branched-chain amino
acids (leucine, isoleucine, and valine). The reaction catalyzed by the BCKDH complex is
considered the rate-limiting step for the catabolism of all three of the branched-chain amino …
