Which amino acids accumulate in maple syrup urine disease?
AValine
BLeucine
CIsoleucine
DAll branched-chain amino acids
History of dislike for sweet food items is typically present in:
AGlycogen storage disease
BDiabetes mellitus
CGalactosemia
DHereditary fructose intolerance
Which enzyme is deficient in Galactosemia?
AHexosaminidase B
BHexosaminidase A
CGalactose 1-phosphate uridyltransferase
DGlucocerebrosidase
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