Results 211 to 220 of about 155,562 (237)

Effects of tetrahydrobiopterin and phenylalanine on in vivo human phenylalanine hydroxylase by phenylalanine breath test

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, Minoru Asada, Yoshiyuki Okano, Kazuhiko Takatori, Tsunekazu Yamano, Masahiro Kajiwara, Satoshi Kudo   +6 more
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Activation of phenylalanine hydroxylase by phenylalanine

Biochimica et Biophysica Acta (BBA) - Enzymology, 1971
Abstract 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.
openaire   +3 more sources

THE EFFECTS OF PHENYLALANINE ANALOGUES ON THE METABOLISM OF PHENYLALANINE IN RATS

Canadian Journal of Biochemistry, 1967
Rats 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, 1972
Abstract 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, J. A. Völlmin, H.-Ch. Curtius   +2 more
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Determination of p-halogenated phenylalanines and phenylalanine in plasma

Analytical Biochemistry, 1971
Abstract A method for the determination, either in combination or singularly, for phenylalanine or some p -halogenated phenylalanines in blood plasma is presented. A thin-layer chromatographic procedure was elaborated which separates the p -halogenated phenylalanines from phenylalanine by means of a cation-exchange resin.
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Fluorescence of the fluorinated phenylalanines

Analytical Biochemistry, 1962
Abstract Fluorinated phenylalanines are much more intensely fluorescent than phenylalanine or the corresponding chlorinated or brominated compounds. The p -derivative fluoreces with a quantum efficiency of 0.40. These compounds possess excitation maxima at about 268 mμ and fluoresce at about 292 mμ.
Gordon Guroff, Michael A. Chirigos
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Phenylalanine Hydroxylase Activity

Archives of Pediatrics & Adolescent Medicine, 1983
Sir .—In the article, "Diagnosis of Phenylalanine Hydroxylase Deficiency (Phenylketonuria)" (Journal1982;136:111-114), we are concerned by the recommendation of Berry and collegues that centers treating patients with phenylketonuria (PKU) should " . . .
David M. Danks, Richard G.H. Cotton
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Adsorption chromatography of phenylalanine

Biotechnology and Bioengineering, 1989
Utilisation de la resine Amberlite XAD-4 dans le but de retenir des acides amines aromatiques tels que la phenylalanine, a partir de l'hydrolyse acide de la caseine.
Ana Lúcia Coutinho Santos Neto, Ana Maria H. Vasconcellos, Cesário Paulo Honório de Oliveira, Denise Mattatia Grassiano   +3 more
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Phenylalanine hydroxylase deficiency

Genetics in Medicine, 2011
Phenylalanine hydroxylase deficiency is an autosomal recessive disorder that results in intolerance to the dietary intake of the essential amino acid phenylalanine. It occurs in approximately 1:15,000 individuals. Deficiency of this enzyme produces a spectrum of disorders including classic phenylketonuria, mild phenylketonuria, and mild ...
Yannis Trakadis, Charles R. Scriver, John J. Mitchell   +2 more
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Characterization of phenylalanine hydroxylase

Biochemistry, 1986
Iron can be bound to phenylalanine hydroxylase (PAH) in two environments. The assignment of the electron paramagnetic resonance spectrum of PAH to two, overlapping high-spin ferric signals is confirmed by computer simulation. Both environments are shown to be populated in the crude enzyme.
L. M. Bloom, Stephen J. Benkovic, Betty J. Gaffney   +2 more
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