What does the branched chain alpha-keto acid dehydrogenase do?
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The BCKD enzyme complex is responsible for one step in the normal breakdown of three protein building blocks (amino acids). These amino acids—leucine, isoleucine, and valine—are obtained from the diet. They are present in many kinds of food, particularly protein-rich foods such as milk, meat, and eggs.
What are the cofactors in the branched chain alpha-keto acid dehydrogenase complex?
This enzyme complex is composed of three catalytic components: alpha-ketoacid dehydrogenase (also referred to as the E1 component), dihydrolipoyl transacylase (E2 component), and dihydrolipoamide dehydrogenase (E3 component).
Which enzymes have an alpha-keto acid substrate?
Only pyruvate dehydrogenase and α-ketoglutarate dehydrogenase have a ketoacid substrate (pyruvate and α-ketoglutarate, respectively). Both these enzymes are large complexes that require five cofactors, and produce a CoA thiol ester.
What causes maple syrup urine disease?
Causes. Maple syrup urine disease (MSUD) is inherited, which means it is passed down through families. It is caused by a defect in 1 of 3 genes. People with this condition cannot break down the amino acids leucine, isoleucine, and valine.
What is the function of alpha-ketoglutarate dehydrogenase?
Alpha-ketoglutarate dehydrogenase (α-KGDH) is a highly regulated enzyme, which could determine the metabolic flux through the Krebs cycle. It catalyses the conversion of α-ketoglutarate to succinyl-CoA and produces NADH directly providing electrons for the respiratory chain.
Where is Alpha-ketoglutarate dehydrogenase located?
mitochondrial matrix
This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO2 during the citric acid cycle. The protein is located in the mitochondrial matrix and uses thiamine pyrophosphate as a cofactor.
What is branched-chain alpha-keto acid dehydrogenase complex?
Branched-chain alpha-keto acid dehydrogenase complex. The branched-chain α-ketoacid dehydrogenase complex ( BCKDC or BCKDH complex) 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.
What are the three components of the alpha ketoacid dehydrogenase enzyme complex?
This enzyme complex is composed of three catalytic components: alpha-ketoacid dehydrogenase (also referred to as the E 1 component), dihydrolipoyl transacylase (E 2 component), and dihydrolipoamide dehydrogenase (E 3 component).
What is the role of BCKDH kinase in the catabolic pathway?
Branched-chain alpha-keto acid dehydrogenase (BCKDH) complex catalyzes the committed step of branched-chain amino acid catabolism, and its activity is regulated by the phosphorylation-dephosphorylation cycle. BCKDH kinase is responsible for inactivation of the complex by phosphorylation. In the pres …
What is the function of BCKDHA?
BCKDHA branched chain keto acid dehydrogenase E1, alpha polypeptide [ (human)] The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine.