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Myofibrillar Protein Synthesis Rates Do not Differ With Low and High Estradiol Concentrations Across the Menstrual Cycle.

J Clin Endocrinol Metab
Apicella MCA, Jameson TSO, Monteyne AJ, Pavis GF, Abdelrahman DR, Murton AJ, Alamdari N, Dirks ML, Wall BT, Stephens FB.   +9 more
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Phenylalanine hydroxylase mutations and phenylalanine-tyrosine metabolism in heterozygotes for phenylalanine hydroxylase deficiency

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, E. Riva, C. Agostoni, S. Leviti, L. Fiori, A.M. Lammardo, M. L. Biondi, M. Giovannini   +7 more
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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 " . . .
D M, Danks, R G, Cotton
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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 ...
John J, Mitchell, Yannis J, Trakadis, Charles R, Scriver   +2 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.
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Phenylalanine supplementation improves the phenylalanine profile in tyrosinaemia

Journal of Inherited Metabolic Disease, 2000
AbstractTyrosinaemia 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.
C J, Wilson, K G, Van Wyk, J V, Leonard, P T, Clayton   +3 more
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Phenylalanine deficiency syndrome

The Journal of Pediatrics, 1966
Clinical observations are recorded on two infants in whom failure to gain, eczema and listlessness developed during severe dietary restriction of phenylalanine. Regular diet was resumed when they were found not to have phenylketonuria; in spite of improvement in their nutritional status, their developmental progress remained slow.
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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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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
Yoshiyuki, Okano, Kazuhiko, Takatori, Satoshi, Kudo, Tomoko, Sakaguchi, Minoru, Asada, Masahiro, Kajiwara, Tsunekazu, Yamano   +6 more
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Brominated phenylalanines

Bulletin des Sociétés Chimiques Belges, 1974
Abstract(3‐(3‐Bromo‐4‐hydroxy‐S‐methoxy‐phenyl)alanine (Ie) exerts a long‐acting antihypertensive effect in rats and dogs : its synthesis and its optical resolution have already appeared in the literature. 1 Ten new mono‐ and polybrominated phenylalanines with hydroxy‐ and/or methoxy‐groups are here described.
M. L. Anhoury, P. Crooy, R. De Neys, J. Eliaers   +3 more
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