<prompt> All of the following are excreted in urine in Maple syrup urine disease, EXCEPT:LeucineIsoleucineValineNone of the above  <response>: 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. 