<prompt> Maple syrup urine disease is due accumulation ofPhenylalanineTyrosineBranched chain amino acidsTryptophan  <response>: Maple syrup urine disease is also called branched-chain ketonuria The basic defect is deficient decarboxylation of branched-chain ketoacids.Ref: DM Vasudevan - Textbook of Biochemistry, 7th edition, page no: 230 