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Biochemistry General 420bc4b2

Branched chain ketoacid decarboxylation is defective in

A
Maple Syrup urine disease
B
Hartnup disease
C
Alkaptonuria
D
GMI Gangliosidosis
High-Yield Explanation
Maple syrup urine disease is due to deficiency of the enzyme branched chain α-keto dehydrogenase which catalyzes decarboxylation of branched - chain amino acid.

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