Which of the following amino acid is excreted in urine in maple syrup urine disease: (A) Tryptophan, (B) Phenylalanine, (C) Leucine, (D) Arginine
Answer is C. In maple syrup urine disease there is the excretion of branched chain amino acids (isoleucine, leucine, valine) and their keto acids (α -keto β-methyl valerate, α-ketoisocaproate, α-ketoisovalerate) in the urine.