<prompt> In maple syrup disease, the amino acids excreted in urine are, EXCEPT:ValineLeucineIsoleucinePhenylalanine  <response>: Maple syrup urine disease MSUD, or branched-chain ketoaciduria, is caused by a deficiency in activity of the branched-chain -keto acid dehydrogenase Inborn error of metabolism of valine leucine and isoleucine associated with gross mental deficiency. Branched chain alpha-keto acid dehydrogenase is located in the inner mitochondrial membrane and requires thiamin pyrophosphate, lipoic acid, CoA, and Nicotinamide adenine dinucleotide NAD Disease associated with Branched chain aminoacids: Disease Deficiency Maple syrup urine disease Alpha keto acid deydrogenase deficiency Isovaleic acidaemia IsovalerylCoA dehydrogenase deficiency Methylmanlonic acidaemia Coenzyme A mutase deficiency Methyl malonyl CoA mutase deficieny Propionic acidaemia Propionyl CoA carboxylase deficiency Leucinosis Isoleucine and lecine transaminase deficiency Valinaemia Valine dehydrogenase NADPdeficiency Ref: Harpers illustrated biochemistry, 26 th Edition, Page 259 