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Inactivation of purified phenylalanine hydroxylase by dithiothreitol
Biochemical and Biophysical Research Communications, 1992Purified rat liver phenylalanine hydroxylase is inactivated in vitro by ascorbate and thiol compounds, dithiothreitol being the most effective inhibitor, with a second order rate constant for the inactivation of 0.066 +/- 0.002 mM-1.min-1 at 20 degrees C and pH 7.2.
Torgeir Flatmark +3 more
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Hepatic Phenylalanine Hydroxylase and PKU
1975The conversion of phenylalanine to tyrosine in mammalian tissues is catalyzed by a complex enzyme system composed of several essential enzymes and cofactors. All of these components have been assayed in liver biopsy samples from patients with the classic form of PKU.
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Phenylalanine Hydroxylase Activity-Reply
Archives of Pediatrics & Adolescent Medicine, 1983In Reply .—For most metabolic disorders, a diagnosis based on a single symptom would not be acceptable. Hyperphenylalaninemia is a symptom that in a newborn infant suggests either complete or partial PH deficiency (PKU), dihydropteridine reductase deficiency, or deficiency in biopterin synthesis.
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A direct assay for liver phenylalanine hydroxylase
Biochimica et Biophysica Acta (BBA) - Enzymology, 1969Abstract 1. 1 An assay for liver phenylalanine hydroxylase ( l -phenylalanine, tetrahydropteridine: O2 oxidoreductase (4-hydroxylating), EC 1.14.3.1) based on the nonenzymatic regeneration of 2-amino-4-hydroxy-6,7-dimethyltetrahydropteridine from 2-amino-4-hydroxy-6,7-dimethyldihydropteridine (quinonoid form) by dithiothreitol is described ...
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On the mechanism of action of phenylalanine hydroxylase
Biochemical Society Transactions, 1985Thomas A. Dix +6 more
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Regulation of the activity of hepatic phenylalanine hydroxylase
Advances in Enzyme Regulation, 1986Rat liver phenylalanine hydroxylase catalyzes the tetrahydropterin-dependent oxidation of phenylalanine to tyrosine, according to equation 1. In addition to the naturally-occurring coenzyme, tetrahydrobiopterin (BH4), certain synthetic analogs of BH4 such as 6-methyltetrahydropterin (6MPH4) have high cofactor activity.
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Effects of phenylalanine on phenylalanine hydroxylase separation and stability
Biochemical Medicine, 1985Ennis C. Layne +2 more
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[74] Phenylalanine hydroxylase (Pseudomonas)
1970Publisher Summary This chapter discusses the assay, purification, and properties of phenylalanine hydroxylase from Pseudomonas. The hydroxylation of phenylalanine to tyrosine is catalyzed by an inducible enzyme from Pseudomonas. This conversion requires molecular oxygen and a tetrahydropteridine.
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The Mechanism of Action of Phenylalanine Hydroxylase
Annals of the New York Academy of Sciences, 1986Stephen J. Benkovic +5 more
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Phenylalanine Hydroxylase,Tyrosine Hydroxylase,Tryptophan Hydroxylase
2003Corinne Abate, Tong H. Joh, Onyou Hwang
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