branched-chain α-keto acid dehydrogenase enzyme complex(English)

• catalyzes the second, irreversible step in BCAA metabolism, which is oxidative decarboxylation of the branched-chain α-keto acid (BCKA) products of the BCAT reaction. Maple Syrup Urine Disease (MSUD) results from genetic defects in BCKDC
• MSUD, BCAT, BCKA, BCAA
• Enzymes
• https://doi.org…nana.2012.00018