phenylalanine 4-monooxygenase activity

Known as: phenylalaninase activity, L-phenylalanine,tetrahydrobiopterin:oxygen oxidoreductase (4-hydroxylating), PAH activity 
Catalysis of the reaction: L-phenylalanine + tetrahydrobiopterin + O2 = L-tyrosine + 4-alpha-hydroxytetrahydrobiopterin. [EC:1.14.16.1]
National Institutes of Health

Topic mentions per year

Topic mentions per year

1962-2018
051019622018

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Highly Cited
2008
Highly Cited
2008
Phenylketonuria (PKU) is an inborn error of metabolism caused by mutations in phenylalanine hydroxylase (PAH). Over 500 disease… (More)
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2008
2008
Mutations in the phenylalanine hydroxylase (PAH) gene result in phenylketonuria (PKU). Tetrahydrobiopterin (BH(4))-responsive… (More)
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Highly Cited
2007
Highly Cited
2007
Phenylketonuria (PKU; OMIM 261600) is an autosomal recessive inborn error of phenylanaline metabolism. PKU is characterized by… (More)
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2006
2006
Novel recombinant adeno-associated virus vectors pseudotyped with serotype 8 capsid (rAAV2/8) have recently shown exciting… (More)
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2006
2006
Phenylketonuria (PKU) is the most frequent disturbance of amino acid metabolism being caused by severe deficiency of… (More)
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2004
2004
A subtype of phenylalanine hydroxylase (PAH) deficiency that responds to cofactor (tetrahydrobiopterin, BH4) supplementation has… (More)
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2004
2004
The natural cofactor of phenylalanine hydroxylase (PAH), tetrahydrobiopterin (BH4), regulates the enzyme activity as well as… (More)
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2004
2004
Purpose: Phenylketonuria (PKU) is an autosomal recessive disorder caused by mutations in the phenylalanine hydroxylase (PAH) gene… (More)
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1997
1997
To determine if there is abnormal phenylalanine and biopterin metabolism in patients with dopa-responsive dystonia (DRD), we… (More)
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1990
1990
Mutant mice exhibiting heritable hyperphenylalaninemia have been isolated after ethylnitrosourea mutagenesis of the germ line. We… (More)
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