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Tag: Ketoaciduria

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What are branched-chain amino acids in urine?

4 min read
According to the National Organization for Rare Disorders, Maple Syrup Urine Disease (MSUD) affects approximately 1 in 185,000 babies worldwide. Excess branched-chain amino acids in urine, which give it a distinct sweet odor, are a hallmark of this inherited metabolic disorder, caused by a genetic inability to properly break down leucine, isoleucine, and valine.