RT Book, Section A1 King, Michael W. SR Print(0) ID 1122104375 T1 Nitrogen: Amino Acid Metabolism T2 Integrative Medical Biochemistry Examination and Board Review YR 2014 FD 2014 PB McGraw-Hill Education PP New York, NY SN 9780071786126 LK accesspharmacy.mhmedical.com/content.aspx?aid=1122104375 RD 2024/03/28 AB High-Yield TermsGlucogenic: relating to amino acids whose catabolic products can be used for net glucose synthesis during gluconeogenesisKetogenic: relating to amino acids whose catabolic products enter the tricarboxylic acid (TCA) cycle but cannot be used for net glucose synthesis during gluconeogenesisEssential amino acid: any amino acid that cannot be formed de novo or from other precursor compounds within mammalian cellsGlutamate dehydrogenase: critical enzyme catalyzing the interconversion of glutamate and α-ketoglutarate, serves as a gateway reaction between cellular energy demands and nitrogen homeostasisAsparaginase: enzyme catalyzing the deamination of asparagine to aspartate, enzyme is used clinically as a chemotherapeutic treatment for certain leukemiasCystathionine β-synthase: enzyme involved in cysteine biosynthesis, deficiencies are most common cause of homocystinuriasGlycine cleavage complex: also called glycine decarboxylase, enzyme-catabolizing glycine, defects in activity cause glycine encephalopathyPhenylketonuria (PKU): a particular form of hyperphenylalaninemia resulting from defects in the phenylalanine hydroxylase geneBranched-chain amino acid: consists of leucine, isoleucine, and lysine; defects in the catabolic enzyme branched-chain keto acid dehydrogenase (BCKD) result in Maple syrup urine disease (MSUD)Alkaptonuria: first inherited error in metabolism to be characterized, benign disease caused by defects in the tyrosine-catabolizing enzyme homogentisate oxidase, urine of afflicted patients turns brown on exposure to air