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Subjects/Biochemistry/Amino Acidurias — PKU, Alkaptonuria, Maple Syrup

Amino Acidurias — PKU, Alkaptonuria, Maple Syrup

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flask-conical Biochemistry

Which of the following amino acidurias is characterized by accumulation of branched-chain amino acids and their ketoacid metabolites, with a distinctive sweet-smelling urine?

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