8-423-08AASynth - problem with the Phe hydroxylase HPA One...

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1 Or, gluconeogenesis, FA synthesis AA breakdown AA synthesis
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2 AA synthesis overview: -all are derived from intermediates of glycolysis, CAC, or PPP -Nitrogen enters by way of glutamate and glutamine -“families” of aas from six precursors -transamination reactions common -1-Carbon transfers common Serine, glycine synthesis -glycolytic precursor ( # ) -glutamate the N-donor, in a transamination reaction (<-----) -H 4 folate used to remove a 1-C group ( * ) (#from glycolysis) <----- *
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3 Transfers 1-C groups in intermediate oxidation states Transfers 1-C groups in most reduced state- methyl groups CH 4 Transfers 1-C groups in most oxidized state-CO 2 An essential aa Tyrosine synthesis -derived from the essential aa phenylalanine -Phe hydroxylase a mixed function oxidase
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4 What if there is a
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Unformatted text preview: problem with the Phe hydroxylase? Hyperphenylalaninemia HPA One severe type of HPA is Phenylketonuria PKU X Excessive levels of Phe build up Accumulation of aromatic metabolites of Phe in neural tissues 5 Excessive levels of Phe build up Accumulation of aromatic metabolites of Phe in neural tissues:-severe mental retardation In PKU: These compounds end up in urine, imparting a “mousy” odor 6 Glutamine made from glutamate and NH 4 + ; needs ATP Very highly regulated; it is the central entry point for nitrogen into metabolism Both allosteric modulation and reversible covalent modification used to regulate glutamine synthetase 7 Allosteric regulation Reversible covalent regulation...
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This note was uploaded on 04/05/2008 for the course BIOCHEM 423 taught by Professor Osgood during the Spring '08 term at New Mexico.

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8-423-08AASynth - problem with the Phe hydroxylase HPA One...

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