Which enzyme's deficiency leads to the development of maple syrup urine disease?

A Branched-chain alpha-keto acid dehydrogenase complex ✓

B Phenylalanine hydroxylase

C Tyrosinase

D Homogentisate 1,2-dioxygenase

Explanation

Maple syrup urine disease is caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex, leading to the accumulation of branched-chain amino acids and their toxic metabolites.

Source

NCBI: Maple Syrup Urine Disease

Enzymes in Disease Biochemistry Amino Acid Metabolism Disorders Medium MEDICAL Confidence: high

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Normal Values of Immunology Parameters

Which enzyme's deficiency leads to the development of maple syrup urine disease?

Explanation

Source

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