Which enzyme's dysfunction is associated with maple syrup urine disease?

A Pyruvate kinase
B Branched-chain alpha-keto acid dehydrogenase complex ✓
C Fructose-1,6-bisphosphatase
D Gluconease

Explanation

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

Source

OMIM Database
Enzymes in Disease Genetics Inborn Errors of Metabolism Hard MEDICAL Confidence: high