Which enzyme deficiency is responsible for the development of Maple Syrup Urine Disease (MSUD)?

A Branched-chain alpha-keto acid dehydrogenase complex ✓

B Phenylalanine hydroxylase

C Tyrosinase

D Galactose-1-phosphate uridyltransferase

Explanation

Maple Syrup Urine Disease (MSUD) is caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex, which is necessary for the breakdown of certain amino acids.

Source

Nelson's Textbook of Pediatrics

Metabolic and Nutritional Disorders Genetic Metabolic Disorders Amino Acid Disorders Hard MEDICAL Confidence: high

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Which enzyme deficiency is responsible for the development of Maple Syrup Urine Disease (MSUD)?

Explanation

Source

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