4
The degradation of branched chain amino acids predominantly occurs in___, and the first step of removing the
group is catalysed by enzyme
EXPLANATION
Protein Engineering, Disulphide Bond Reductio
- Subcellular Site and Enzymology:
- The branched-chain amino acids (Leucine, Isoleucine, Valine) are essential amino acids whose primary catabolism occurs in extrahepatic tissues and hepatocytes within the mitochondria.
- First Step (Transamination):
- The $\alpha$-amino group is removed and transferred to $\alpha$-ketoglutarate by the enzyme branched-chain amino acid aminotransferase (transaminase), yielding glutamate and the corresponding branched-chain $\alpha$-keto acids.
- Second Step (Oxidative Decarboxylation):
- Catalyzed by the mitochondrial branched-chain $\alpha$-keto acid dehydrogenase (BCKDH) complex.
- nalysis:
- (mitochondria; amino; transaminase): Correct.
Correct option: (3) mitochondria; amino; transaminase
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