RT Book, Section A1 Rodwell, Victor W. A2 Rodwell, Victor W. A2 Bender, David A. A2 Botham, Kathleen M. A2 Kennelly, Peter J. A2 Weil, P. Anthony SR Print(0) ID 1162228887 T1 Catabolism of the Carbon Skeletons of Amino Acids T2 Harper's Illustrated Biochemistry, 31e YR 2018 FD 2018 PB McGraw-Hill Education PP New York, NY SN 9781259837937 LK accesspharmacy.mhmedical.com/content.aspx?aid=1162228887 RD 2024/03/28 AB OBJECTIVESAfter studying this chapter, you should be able to:Name the principal catabolites of the carbon skeletons of the protein amino acids and the major metabolic fates of these catabolites.Write an equation for an aminotransferase (transaminase) reaction and illustrate the role played by the coenzyme.Outline the metabolic pathways for each of the protein amino acids, and identify reactions associated with clinically significant metabolic disorders.Provide examples of aminoacidurias that arise from defects in glomerular tubular reabsorption, and the consequences of impaired intestinal absorption of tryptophan.Explain why metabolic defects in different enzymes of the catabolism of a specific amino acid can be associated with similar clinical signs and symptoms.Describe the implications of a metabolic defect in Δ1-pyrroline-5-carboxylate dehydrogenase for the catabolism of proline and of 4-hydroxyproline.Explain how the α-amino nitrogen of proline and of lysine is removed by processes other than transamination.Draw analogies between the reactions that participate in the catabolism of fatty acids and of the branched-chain amino acids.Identify the specific metabolic defects in hypervalinemia, maple syrup urine disease, intermittent branched-chain ketonuria, isovaleric acidemia, and methylmalonic aciduria.