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THE ENZYMATIC CONVERSION OF PHENYLALANINE TO TYROSINE
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Acta Paediatrica, 2002
The aim of this study was to determine whether any relationship exists between the severity of mutation of the phenylalanine hydroxylase (PAH) gene and the plasma concentrations of phenylalanine (Phe) and tyrosine (Tyr) under fasting and semifasting conditions among heterozygotes in a matched case-control study.
E. Verduci+7 more
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The aim of this study was to determine whether any relationship exists between the severity of mutation of the phenylalanine hydroxylase (PAH) gene and the plasma concentrations of phenylalanine (Phe) and tyrosine (Tyr) under fasting and semifasting conditions among heterozygotes in a matched case-control study.
E. Verduci+7 more
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Redesign of a Phenylalanine Aminomutase into a Phenylalanine Ammonia Lyase
ChemCatChem, 2013AbstractAn aminomutase, naturally catalyzing the interconversion of (S)‐α‐phenylalanine and (R)‐β‐phenylalanine, was converted into an ammonia lyase catalyzing the nonoxidative deamination of phenylalanine to cinnamic acid by a rational single‐point mutation.
Sebastian Bartsch+6 more
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Isozymes of Phenylalanine Hydroxylase [PDF]
Three isozymes of phenylalanine hydroxylase exist in adult rat liver. They are chromatographically unique. Partial characterization suggests that they are similar in chemical properties and differ only in charge. Estimation of the Stokes radii indicates that the isozymes have similar molecular weights of about 200,000. Two isozymes exist in human fetal
Paul J. Geiger+3 more
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Phenylalanine supplementation improves the phenylalanine profile in tyrosinaemia
Journal of Inherited Metabolic Disease, 2000AbstractTyrosinaemia types I and II are caused by enzyme deficiencies in the tyrosine catabolism pathway. Successful treatment is possible with the novel enzyme inhibitor NTBC in tyrosinaemia type I and with dietary tyrosine and phenylalanine restriction in both conditions.
J. V. Leonard+3 more
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Molecular Genetics and Metabolism, 2007
BH(4) administration results in the reduction of blood phenylalanine level in patients with tetrahydrobiopterin (BH(4))-responsive phenylalanine hydroxylase (PAH) deficiency. The mechanism underlying BH(4) response remains unknown. Here, we studied the effects of BH(4) and phenylalanine on in vivo PAH activity of normal controls using the phenylalanine
Tomoko Sakaguchi+6 more
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BH(4) administration results in the reduction of blood phenylalanine level in patients with tetrahydrobiopterin (BH(4))-responsive phenylalanine hydroxylase (PAH) deficiency. The mechanism underlying BH(4) response remains unknown. Here, we studied the effects of BH(4) and phenylalanine on in vivo PAH activity of normal controls using the phenylalanine
Tomoko Sakaguchi+6 more
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Activation of phenylalanine hydroxylase by phenylalanine
Biochimica et Biophysica Acta (BBA) - Enzymology, 1971Abstract 1. 1. Phenylalanine activates phenylalanine hydroxylase ( l -phenylalanine, tetrahydropteridine:oxygen oxidoreductase (4-hydroxylating), EC 1.14.3.1), when dithiothreitol is used to regenerate 2-amino-4-hydroxy-6,7-dimethyltetrahydropteridine. 2. 2.
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THE EFFECTS OF PHENYLALANINE ANALOGUES ON THE METABOLISM OF PHENYLALANINE IN RATS
Canadian Journal of Biochemistry, 1967Rats expired14CO2slightly faster when DL-phenylalanine-1-14C rather than DL-phenylalanine-3-14C was injected. An injection of DL-fluorophenylalanine-3-14C produced more radioactive CO2than did phenylalanine-3-14C; DL-beta-thienylalanine-3-14C produced only trace amounts of14CO2but significant levels of radioactivity in the urine.
Gail Dolan, Claude Godin
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The use of deuterated phenylalanine for the elucidation of the phenylalanine-tyrosine metabolism
Clinica Chimica Acta, 1972Abstract The application in vivo of stable isotopes, e.g. deuterium, for the study of metabolic pathways is demonstrated on the example of the phenylalanine-tyrosine metabolism. A healthy child, a patient with phenylketonuria and a patient with hyper phenylalaninemia were loaded with 200 mg/kg of deuterated phenylalanine.
K. Baerlocher+2 more
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