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What is All of the following are excreted in urine in Maple syrup urine disease, EXCEPT: and explain in detail?
As the name implies, the odor of urine in maple syrup urine disease (branched-chain ketonuria) suggests maple syrup or burnt sugar. The biochemical defect involves the -keto acid decarboxylase complex. Plasma and urinary levels of leucine, isoleucine, valine, -keto acids, and -hydroxy acids (reduced -keto acids) are elevated. Ref: Harper 28th edition, chapter 29.