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Phenylalanine Hydroxylase

Known as: Phenylalanine 4 Hydroxylase, 4-Hydroxylase, Phenylalanine, phenylalaninase 
An enzyme of the oxidoreductase class that catalyzes the formation of L-TYROSINE, dihydrobiopterin, and water from L-PHENYLALANINE… Expand
National Institutes of Health

Papers overview

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Review
2011
Review
2011
Phenylalanine hydroxylase deficiency is an autosomal recessive disorder that results in intolerance to the dietary intake of the… Expand
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Highly Cited
2009
Highly Cited
2009
Hydrogels can be prepared using the commercially available Fmoc-phenylalanine or Fmoc-tyrosine as the gelator. Gelation is… Expand
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Highly Cited
2008
Highly Cited
2008
Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resulting from deficiency of… Expand
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Highly Cited
2008
Highly Cited
2008
Mutations in the phenylalanine hydroxylase (PAH) gene result in phenylketonuria (PKU). Tetrahydrobiopterin (BH(4))-responsive… Expand
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Highly Cited
2004
Highly Cited
2004
Phenylketonuria patients harboring a subset of phenylalanine hydroxylase (PAH) mutations have recently shown normalization of… Expand
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Highly Cited
1999
Highly Cited
1999
Abstract Serum phenylalanine concentrations decreased in 4 patients with hyperphenylalaninemia after loading with… Expand
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Review
1999
Review
1999
Phenylalanine hydroxylase, tyrosine hydroxylase, and tryptophan hydroxylase constitute a small family of monooxygenases that… Expand
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Highly Cited
1999
Highly Cited
1999
Phenylalanine hydroxylase converts phenylalanine to tyrosine, a rate-limiting step in phenylalanine catabolism and protein and… Expand
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Highly Cited
1998
Highly Cited
1998
Phenylketonuria (PKU) and mild hyperphenylalaninemia (MHP) are allelic disorders caused by mutations in the gene encoding… Expand
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Highly Cited
1996
Highly Cited
1996
HNF1 is a transcriptional activator of many hepatic genes including albumin, alpha1-antitrypsin, and alpha- and beta-fibrinogen… Expand
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