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Reference on EC 1.14.16.1 - phenylalanine 4-monooxygenase

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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Kaufman, S.
Aromatic amino acid hydroxylases
The Enzymes, 3rd Ed. (Boyer, P. D. , ed. )
18
217-282
1987
Homo sapiens, Rattus norvegicus
-
Manually annotated by BRENDA team
Koizumi, S.; Matsushima, Y.; Nagatsu, T.; Linuma, H.; Takeuchi, T.; Umezawa, H.
3,4-Dihydroxystyrene, a novel microbial inhibitor for phenylalanine hydroxylase and other pteridine-dependent monooxygenases
Biochim. Biophys. Acta
789
111-118
1984
Rattus norvegicus
Manually annotated by BRENDA team
Daubner, S.C.; Melendez, J.; Fitzpatrick, P.F.
Reversing the substrate specificities of phenylalanine and tyrosine hydroxylase: aspartate 425 of tyrosine hydroxylase is essential for L-DOPA formation
Biochemistry
39
9652-9661
2000
Rattus norvegicus
Manually annotated by BRENDA team
Kaufman, S.
Phenylalanine 4-monooxygenase from rat liver
Methods Enzymol.
142
3-17
1987
Rattus norvegicus
Manually annotated by BRENDA team
Shiman, R.
Purification and assay of rat liver phenylalanine 4-monooxygenase
Methods Enzymol.
142
17-27
1987
Rattus norvegicus
Manually annotated by BRENDA team
Abita, J.P.; Blandin-Savoja, F.; Rey, F.
Phenylalanine 4-monooxygenase from human liver
Methods Enzymol.
142
27-35
1987
Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Doskeland, A.P.; Doskeland, S.O.; Flatmark, T.
Phenylalanine 4-monooxygenase from bovine liver
Methods Enzymol.
142
35-44
1987
Bos taurus
Manually annotated by BRENDA team
Fujisawa, H.; Nakata, H.
Phenylalanine 4-monooxygenase from Chromobacterium violaceum
Methods Enzymol.
142
44-49
1987
Chromobacterium violaceum
Manually annotated by BRENDA team
Pember, S.O.; Villafranca, J.J.; Benkovic, S.J.
Chromobacterium violaceum phenylalanine 4-monooxygenase
Methods Enzymol.
142
50-56
1987
Chromobacterium violaceum
Manually annotated by BRENDA team
Guroff, G.; Rhoads, C.A.
Phenylalanine hydroxylation by Pseudomonas species (ATCC 11299a). Nature of the cofactor
J. Biol. Chem.
244
142-146
1969
Pseudomonas sp.
Manually annotated by BRENDA team
Nakata, H.; Fujisawa, H.
Purification and characterization of phenylalanine 4-monooxygenase from rat liver
Biochim. Biophys. Acta
614
313-327
1980
Rattus norvegicus
Manually annotated by BRENDA team
Al-Janabi, J.M.
Purification of rat liver phenylalanine hydroxylase by affinity chromatography
Arch. Biochem. Biophys.
200
603-608
1980
Rattus norvegicus
Manually annotated by BRENDA team
Yamashita, M.; Minato, S.; Arai, M.; Kishida, Y.; Nagatsu, T.; Umezawa, H.
Purification of phenylalanine hydroxylase from human adult and foetal livers with a monoclonal antibody
Biochem. Biophys. Res. Commun.
133
202-207
1985
Homo sapiens
Manually annotated by BRENDA team
Martinez, A.; Andersson, K.K.; Haavik, J.; Flatmark, T.
EPR and 1H-NMR spectroscopic studies on the paramagnetic iron at the active site of phenylalanine hydroxylase and its interaction with substrates and inhibitors
Eur. J. Biochem.
198
675-682
1991
Bos taurus, Rattus norvegicus
Manually annotated by BRENDA team
Martinez, A.; Olafsdottir, S.; Haavik, J.; Flatmark, T.
Inactivation of purified phenylalanine hydroxylase by dithiothreitol
Biochem. Biophys. Res. Commun.
182
92-98
1992
Rattus norvegicus
Manually annotated by BRENDA team
Ledley, F.D.; Grenett, H.E.; Woo, S.L.C.
Biochemical characterization of recombinant human phenylalanine hydroxylase produced in Escherichia coli
J. Biol. Chem.
262
2228-2233
1987
Homo sapiens
Manually annotated by BRENDA team
Cotton, R.G.H.; Grattan, P.J.
Phenylalanine hydroxylase of Macaca irus A simple purification by affinity chromatography
Eur. J. Biochem.
60
427-430
1975
Macaca fascicularis
Manually annotated by BRENDA team
Bloom, L.M.; Benkovic, S.J.; Gaffney, B.J.
Characterization of phenylalanine hydroxylase
Biochemistry
25
4204-4210
1986
Rattus norvegicus
Manually annotated by BRENDA team
Pember, S.O.; Villafranca, J.J.; Benkovic, S.J.
Phenylalanine hydroxylase from Chromobacterium violaceum is a copper-containing monooxygenase. Kinetics of the reductive activation of the enzyme
Biochemistry
25
6611-6619
1986
Chromobacterium violaceum
Manually annotated by BRENDA team
Abita, J.P.; Parniak, M.; Kaufman, S.
The activation of rat liver phenylalanine hydroxylase by limited proteolysis, lysolecithin, and tocopherol phosphate. Changes in conformation and catalytic properties
J. Biol. Chem.
259
14560-14566
1984
Rattus norvegicus
Manually annotated by BRENDA team
Nakata, H.; Yamauchi, T.; Fujisawa, H.
Phenylalanine hydroxylase from Chromobacterium violaceum. Purification and characterization
J. Biol. Chem.
254
1829-1833
1979
Chromobacterium violaceum
Manually annotated by BRENDA team
Letendre, C.H.; Dickens, G.; Guroff, G.
Phenylalanine hydroxylase from Pseudomonas sp. (ATCC 11299a). Purification, molecular weight, and influence of tyrosine metabolites on activation and hydroxylation
J. Biol. Chem.
250
6672-6678
1975
Pseudomonas sp.
Manually annotated by BRENDA team
Woo, S.L.C.; Gillam, S.S.; Woolf, L.I.
The isolation and properties of phenylalanine hydroxylase from human liver
Biochem. J.
139
741-749
1974
Homo sapiens
Manually annotated by BRENDA team
Shiman, R.; Gray, D.W.; Hill, M.A.
Regulation of rat liver phenylalanine hydroxylase. I. Kinetic properties of the enzyme's iron and enzyme reduction site
J. Biol. Chem.
269
24637-24646
1994
Rattus norvegicus
Manually annotated by BRENDA team
Shiman, R.; Xia, T.; Hill, M.A.; Gray, D.W.
Regulation of rat liver phenylalanine hydroxylase. II. Substrate binding and the role of activation in the control of enzymic activity
J. Biol. Chem.
269
24647-24656
1994
Rattus norvegicus
Manually annotated by BRENDA team
Kowlessur, D.; Yang, X.J.; Kaufman, S.
Further studies of the role of Ser-16 in the regulation of the activity of phenylalanine hydroxylase
Proc. Natl. Acad. Sci. USA
92
4743-4747
1995
Rattus norvegicus
Manually annotated by BRENDA team
Martinez, A.; Knappskog, P.M.; Olafsdottir, S.; Doskeland, A.P.; Eiken, H.G.; Svebak, R.M.; Bozzini, M.; Apold, J.; Flatmark, T.
Expression of recombinant human phenylalanine hydroxylase as fusion protein in Escherichia coli circumvents proteolytic degradation by host cell proteases. Isolation and characterization of the wild-type enzyme
Biochem. J.
306
589-597
1995
Homo sapiens
-
Manually annotated by BRENDA team
Kappock, T.J.; Harkins, P.C.; Friedenberg, S.; Caradonna, J.P.
Spectroscopic and kinetic properties of unphosphorylated rat hepatic phenylalanine hydroxylase expressed in Escherichia coli. Comparison of resting and activated states
J. Biol. Chem.
270
30532-30544
1995
Rattus norvegicus
Manually annotated by BRENDA team
Carr, R.T.; Balasubramanian, S.; Hawkins, P.C.D.; Benkovic, S.J.
Mechanism of metal-independent hydroxylation by Chromobacterium violaceum phenylalanine hydroxylase
Biochemistry
34
7525-7532
1995
Chromobacterium violaceum, Rattus norvegicus
Manually annotated by BRENDA team
Doeskeland, A.P.; Martinez, A.; Knappskog, P.M.; Flatmark, T.
Phosphorylation of recombinant human phenylalanine hydroxylase: effect on catalytic activity, substrate activation and protection against non-specific cleavage of the fusion protein by restriction protease
Biochem. J.
313
409-414
1996
Homo sapiens
-
Manually annotated by BRENDA team
Kowlessur, D.; Citron, B.A.; Kaufman, S.
Recombinant human phenylalanine hydroxylase: novel regulatory and structural properties
Arch. Biochem. Biophys.
333
85-95
1996
Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Knappskog, P.M.; Flatmark, T.; Aarden, J.M.; Haavik, J.; Martinez, A.
Structure/function relationships in human phenylalanine hydroxylase. Effect of terminal deletions on the oligomerization, activation and cooperativity of substrate binding to the enzyme
Eur. J. Biochem.
242
813-821
1996
Homo sapiens
Manually annotated by BRENDA team
Knappskog, P.M.; Martinez, A.
Effect of mutations at Cys237 on the activation state and activity of human phenylalanine hydroxylase
FEBS Lett.
409
7-11
1997
Homo sapiens
Manually annotated by BRENDA team
Daubner, S.C.; Hillas, P.J.; Fitzpatrick, P.F.
Expression and characterization of the catalytic domain of human phenylalanine hydroxylase
Arch. Biochem. Biophys.
348
295-302
1997
Homo sapiens
Manually annotated by BRENDA team
Chen, D.; Frey, P.A.
Phenylalanine hydroxylase from Chromobacterium violaceum. Uncoupled oxidation of tetrahydropterin and the role of iron in hydroxylation
J. Biol. Chem.
273
25594-25601
1998
Chromobacterium violaceum
Manually annotated by BRENDA team
Teigen, K.; Froystein, N.A.; Martinez, A.
The structural basis of the recognition of phenylalanine and pterin cofactors by phenylalanine hydroxylase: implications for the catalytic mechanism
J. Mol. Biol.
294
807-823
1999
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Gamez, A.; Perez, B.; Ugarte, M.; Desviat, L.R.
Expression analysis of phenylketonuria mutations: effect on folding and stability of the phenylalanine hydroxylase protein
J. Biol. Chem.
275
29737-29742
2000
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Leandro, P.; Rivera, I.; Lechner, M.C.; de Almeida, I.T.; Konecki, D.
The V388M mutation results in a kinetic variant form of phenylalanine hydroxylase
Mol. Genet. Metab.
69
204-212
2000
Homo sapiens
Manually annotated by BRENDA team
Donlon, J.; Fallon, B.; Barrett, P.; Carroll, O.; Henderson, P.; Fairley, J.S.
Hepatic phenylalanine hydroxylase of Clethrionomys glareolus Schreber as a bioindicator of pollution
Biochem. Soc. Trans.
26
S64
1998
Apodemus sylvaticus, Callorhinus ursinus
Manually annotated by BRENDA team
Andersen, O.A.; Flatmark, T.; Hough, E.
High resolution crystal structures of the catalytic domain of human phenylalanine hydroxylase in its catalytically active Fe(II) form and binary complex with tetrahydrobiopterin
J. Mol. Biol.
314
279-291
2001
Homo sapiens
Manually annotated by BRENDA team
Leandro, P.; Lechner, M.C.; Tavares de Almeida, I.; Konecki, D.
Glycerol increases the yield and activity of human phenylalanine hydroxylase mutant enzymes produced in a prokaryotic expression system
Mol. Genet. Metab.
73
173-178
2001
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Bjorgo, E.; De Carvalho, R.M.N.; Flatmark, T.
A comparison of kinetic and regulatory properties of the tetrameric and dimeric forms of wild-type and Thr427->Pro mutant human phenylalanine hydroxylase. Contribution of the flexible hinge region Asp425-Gln429 to the tetramerization and cooperative substrate binding
Eur. J. Biochem.
268
997-1005
2001
Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Andreas Andersen, O.; Flatmark, T.; Hough, E.
Crystal structure of the ternary complex of the catalytic domain of human phenylalanine hydroxylase with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine, and its implications for the mechanism of catalysis and substrate activation
J. Mol. Biol.
320
1095-1108
2002
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Erlandsen, H.; Kim, J.Y.; Patch, M.G.; Han, A.; Volner, A.; Abu-Omar, M.M.; Stevens, R.C.
Structural comparison of bacterial and human iron-dependent phenylalanine hydroxylases: similar fold, different stability and reaction rates
J. Mol. Biol.
320
645-661
2002
Homo sapiens (P00439), Homo sapiens, Chromobacterium violaceum (P30967), Chromobacterium violaceum
Manually annotated by BRENDA team
Ekstrom, F.; Stier, G.; Eaton, J.T.; Sauer, U.H.
Crystallization and X-ray analysis of a bacterial non-haem iron-containing phenylalanine hydroxylase from the Gram-negative opportunistic pathogen Pseudomonas aeruginosa
Acta Crystallogr. Sect. D
59
1310-1312
2003
Pseudomonas aeruginosa
Manually annotated by BRENDA team
Schallreuter, K.U.; Wazir, U.; Kothari, S.; Gibbons, N.C.; Moore, J.; Wood, J.M.
Human phenylalanine hydroxylase is activated by H2O2: a novel mechanism for increasing the L-tyrosine supply for melanogenesis in melanocytes
Biochem. Biophys. Res. Commun.
322
88-92
2004
Homo sapiens
Manually annotated by BRENDA team
Stokka, A.J.; Flatmark, T.
Substrate-induced conformational transition in human phenylalanine hydroxylase as studied by surface plasmon resonance analyses: the effect of terminal deletions, substrate analogues and phosphorylation
Biochem. J.
369
509-518
2003
Homo sapiens
Manually annotated by BRENDA team
Thorolfsson, M.; Teigen, K.; Martinez, A.
Activation of phenylalanine hydroxylase: effect of substitutions at Arg68 and Cys237
Biochemistry
42
3419-3428
2003
Homo sapiens
Manually annotated by BRENDA team
Goreish, A.H.; Bednar, S.; Jones, H.; Mitchell, S.C.; Steventon, G.B.
Phenylalanine 4-monooxygenase and the S-oxidation of S-carboxymethyl-L-cysteine
Drug Metabol. Drug Interact.
20
159-174
2004
Rattus norvegicus, Rattus norvegicus Wistar
Manually annotated by BRENDA team
Infanger, L.C.; Rocheleau, T.A.; Bartholomay, L.C.; Johnson, J.K.; Fuchs, J.; Higgs, S.; Chen, C.C.; Christensen, B.M.
The role of phenylalanine hydroxylase in melanotic encapsulation of filarial worms in two species of mosquitoes
Insect Biochem. Mol. Biol.
34
1329-1338
2004
Armigeres subalbatus (Q5MR21), Armigeres subalbatus, Aedes aegypti (Q8I901), Aedes aegypti
Manually annotated by BRENDA team
Kinzie, S.D.; Thevis, M.; Ngo, K.; Whitelegge, J.; Loo, J.A.; Abu-Omar, M.M.
Posttranslational hydroxylation of human phenylalanine hydroxylase is a novel example of enzyme self-repair within the second coordination sphere of catalytic iron
J. Am. Chem. Soc.
125
4710-4711
2003
Homo sapiens
Manually annotated by BRENDA team
Stokka, A.J.; Carvalho, R.N.; Barroso, J.F.; Flatmark, T.
Probing the role of crystallographically defined/predicted hinge-bending regions in the substrate-induced global conformational transition and catalytic activation of human phenylalanine hydroxylase by single-site mutagenesis
J. Biol. Chem.
279
26571-26580
2004
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Volner, A.; Zoidakis, J.; Abu-Omar, M.M.
Order of substrate binding in bacterial phenylalanine hydroxylase and its mechanistic implication for pterin-dependent oxygenases
J. Biol. Inorg. Chem.
8
121-128
2003
Chromobacterium violaceum (P30967), Chromobacterium violaceum
Manually annotated by BRENDA team
Zoidakis, J.; Sam, M.; Volner, A.; Han, A.; Vu, K.; Abu-Omar, M.M.
Role of the second coordination sphere residue tyrosine 179 in substrate affinity and catalytic activity of phenylalanine hydroxylase
J. Biol. Inorg. Chem.
9
289-296
2004
Chromobacterium violaceum
Manually annotated by BRENDA team
Miranda, F.F.; Kolberg, M.; Andersson, K.K.; Geraldes, C.F.; Martinez, A.
The active site residue tyrosine 325 influences iron binding and coupling efficiency in human phenylalanine hydroxylase
J. Inorg. Biochem.
99
1320-1328
2005
Homo sapiens
Manually annotated by BRENDA team
Zoidakis, J.; Loaiza, A.; Vu, K.; Abu-Omar, M.M.
Effect of temperature, pH, and metals on the stability and activity of phenylalanine hydroxylase from Chromobacterium violaceum
J. Inorg. Biochem.
99
771-775
2005
Chromobacterium violaceum
Manually annotated by BRENDA team
Andersen, O.A.; Stokka, A.J.; Flatmark, T.; Hough, E.
2.0A resolution crystal structures of the ternary complexes of human phenylalanine hydroxylase catalytic domain with tetrahydrobiopterin and 3-(2-thienyl)-L-alanine or L-norleucine: substrate specificity and molecular motions related to substrate binding
J. Mol. Biol.
333
747-757
2003
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Pey, A.L.; Martinez, A.
The activity of wild-type and mutant phenylalanine hydroxylase and its regulation by phenylalanine and tetrahydrobiopterin at physiological and pathological concentrations: An isothermal titration calorimetry study
Mol. Genet. Metab.
86
S43-53
2005
Homo sapiens
Manually annotated by BRENDA team
Pavon, J.A.; Fitzpatrick, P.F.
Insights into the catalytic mechanisms of phenylalanine and tryptophan hydroxylase from kinetic isotope effects on aromatic hydroxylation
Biochemistry
45
11030-11037
2006
Rattus norvegicus
Manually annotated by BRENDA team
Leandro, J.; Nascimento, C.; Tavares de Almeida, I.; Leandro, P.
Co-expression of different subunits of human phenylalanine hydroxylase: Evidence of negative interallelic complementation
Biochim. Biophys. Acta
1762
544-550
2006
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Kim, S.W.; Jung, J.; Oh, H.J.; Kim, J.; Lee, K.S.; Lee, D.H.; Park, C.; Kimm, K.; Koo, S.K.; Jung, S.C.
Structural and functional analyses of mutations of the human phenylalanine hydroxylase gene
Clin. Chim. Acta
365
279-287
2006
Homo sapiens
Manually annotated by BRENDA team
Boonyapiwat, B.; Panagopoulos, P.; Jones, H.; Mitchell, S.C.; Forbes, B.; Steventon, G.B.
Phenylalanine 4-monooxygenase and the S-oxidation of S-carboxymethyl-L-cysteine in HepG2 cells
Drug Metabol. Drug Interact.
21
1-18
2005
Homo sapiens
Manually annotated by BRENDA team
Pey, A.L.; Martinez, A.; Charubala, R.; Maitland, D.J.; Teigen, K.; Calvo, A.; Pfleiderer, W.; Wood, J.M.; Schallreuter, K.U.
Specific interaction of the diastereomers 7(R)- and 7(S)-tetrahydrobiopterin with phenylalanine hydroxylase: implications for understanding primapterinuria and vitiligo
FASEB J.
20
2130-2132
2006
Homo sapiens (P00439)
Manually annotated by BRENDA team
Han, A.Y.; Lee, A.Q.; Abu-Omar, M.M.
EPR and UV-Vis studies of the nitric oxide adducts of bacterial phenylalanine hydroxylase: effects of cofactor and substrate on the iron environment
Inorg. Chem.
45
4277-4283
2006
Chromobacterium violaceum
Manually annotated by BRENDA team
Kozak, L.; Hrabincova, E.; Kintr, J.; Horky, O.; Zapletalova, P.; Blahakova, I.; Mejstrik, P.; Prochazkova, D.
Identification and characterization of large deletions in the phenylalanine hydroxylase (PAH) gene by MLPA: evidence for both homologous and non-homologous mechanisms of rearrangement
Mol. Genet. Metab.
89
300-309
2006
Homo sapiens
Manually annotated by BRENDA team
Lueleyap, H.U.; Alptekin, D.; Pazarba?i, A.; Kasap, M.; Kasap, H.; Demirhindi, H.; Mungan, N.; Ozer, G.; Froster, U.G.
The importance of arginine mutation for the evolutionary structure and function of phenylalanine hydroxylase gene
Mutat. Res.
601
39-45
2006
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Teigen, K.; Jensen, V.R.; Martinez, A.
The reaction mechanism of phenylalanine hydroxylase. - A question of coordination
Pteridines
16
27-34
2005
Chromobacterium violaceum, Homo sapiens, Rattus norvegicus
-
Manually annotated by BRENDA team
Siltberg-Liberles, J.; Martinez, A.
Searching distant homologs of the regulatory ACT domain in phenylalanine hydroxylase
Amino Acids
36
235-249
2008
Homo sapiens
Manually annotated by BRENDA team
Daniele, A.; Cardillo, G.; Pennino, C.; Carbone, M.T.; Scognamiglio, D.; Correra, A.; Pignero, A.; Castaldo, G.; Salvatore, F.
Molecular epidemiology of phenylalanine hydroxylase deficiency in Southern Italy: a 96% detection rate with ten novel mutations
Ann. Hum. Genet.
71
185-193
2007
Homo sapiens
Manually annotated by BRENDA team
Bercovich, D.; Elimelech, A.; Yardeni, T.; Korem, S.; Zlotogora, J.; Gal, N.; Goldstein, N.; Vilensky, B.; Segev, R.; Avraham, S.; Loewenthal, R.; Schwartz, G.; Anikster, Y.
A mutation analysis of the phenylalanine hydroxylase (PAH) gene in the Israeli population
Ann. Hum. Genet.
72
305-309
2008
Homo sapiens
Manually annotated by BRENDA team
Li, J.; Fitzpatrick, P.F.
Characterization of metal ligand mutants of phenylalanine hydroxylase: Insights into the plasticity of a 2-histidine-1-carboxylate triad
Arch. Biochem. Biophys.
475
164-168
2008
Rattus norvegicus
Manually annotated by BRENDA team
Daniele, A.; Cardillo, G.; Pennino, C.; Carbone, M.T.; Scognamiglio, D.; Esposito, L.; Correra, A.; Castaldo, G.; Zagari, A.; Salvatore, F.
Five human phenylalanine hydroxylase proteins identified in mild hyperphenylalaninemia patients are disease-causing variants
Biochim. Biophys. Acta
1782
378-384
2008
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Calvo, A.C.; Pey, A.L.; Ying, M.; Loer, C.M.; Martinez, A.
Anabolic function of phenylalanine hydroxylase in Caenorhabditis elegans
FASEB J.
22
3046-3058
2008
Caenorhabditis elegans (Q9XYQ5), Caenorhabditis elegans
Manually annotated by BRENDA team
Wang, L.; Surendran, S.; Michals-Matalon, K.; Bhatia, G.; Tanskley, S.; Koch, R.; Grady, J.; Tyring, S.K.; Stevens, R.C.; Guttler, F.; Matalon, R.
Mutations in the regulatory domain of phenylalanine hydroxylase and response to tetrahydrobiopterin
Genet. Test.
11
174-178
2007
Homo sapiens
Manually annotated by BRENDA team
Zurflueh, M.R.; Zschocke, J.; Lindner, M.; Feillet, F.; Chery, C.; Burlina, A.; Stevens, R.C.; Thoeny, B.; Blau, N.
Molecular genetics of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency
Hum. Mutat.
29
167-175
2008
Homo sapiens
Manually annotated by BRENDA team
Loaiza, A.; Armstrong, K.M.; Baker, B.M.; Abu-Omar, M.M.
Kinetics of thermal unfolding of phenylalanine hydroxylase variants containing different metal cofactors (FeII, CoII, and ZnII) and their isokinetic relationship
Inorg. Chem.
47
4877-4883
2008
Chromobacterium violaceum (P30967)
Manually annotated by BRENDA team
Leiros, H.K.; Pey, A.L.; Innselset, M.; Moe, E.; Leiros, I.; Steen, I.H.; Martinez, A.
Structure of phenylalanine hydroxylase from Colwellia psychrerythraea 34H, a monomeric cold active enzyme with local flexibility around the active site and high overall stability
J. Biol. Chem.
282
21973-21986
2007
Colwellia psychrerythraea 34H
Manually annotated by BRENDA team
Eavri, R.; Lorberboum-Galski, H.
A novel approach for enzyme replacement therapy. The use of phenylalanine hydroxylase-based fusion proteins for the treatment of phenylketonuria
J. Biol. Chem.
282
23402-23409
2007
Homo sapiens
Manually annotated by BRENDA team
Yan, S.; Wu, G.
Connecting mutant phenylalanine hydroxylase with phenylketonuria
J. Clin. Monit. Comput.
22
333-342
2008
Homo sapiens (P00439)
Manually annotated by BRENDA team
Patel, N.G.; Iliadou, C.; Boonyapiwat, B.; Barlow, D.J.; Forbes, B.; Mitchell, S.C.; Steventon, G.B.
Enzyme kinetic and molecular modelling studies of sulphur-containing substrates of phenylalanine 4-monooxygenase
J. Enzyme Inhib. Med. Chem.
23
958-63
2007
Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Bercovich, D.; Elimelech, A.; Zlotogora, J.; Korem, S.; Yardeni, T.; Gal, N.; Goldstein, N.; Vilensky, B.; Segev, R.; Avraham, S.; Loewenthal, R.; Schwartz, G.; Anikster, Y.
Genotype-phenotype correlations analysis of mutations in the phenylalanine hydroxylase (PAH) gene
J. Hum. Genet.
53
407-418
2008
Homo sapiens
Manually annotated by BRENDA team
Gramer, G.; Burgard, P.; Garbade, S.F.; Lindner, M.
Effects and clinical significance of tetrahydrobiopterin supplementation in phenylalanine hydroxylase-deficient hyperphenylalaninaemia
J. Inherit. Metab. Dis.
30
556-562
2007
Homo sapiens
Manually annotated by BRENDA team
Dobrowolski, S.F.; Ellingson, C.; Coyne, T.; Grey, J.; Martin, R.; Naylor, E.W.; Koch, R.; Levy, H.L.
Mutations in the phenylalanine hydroxylase gene identified in 95 patients with phenylketonuria using novel systems of mutation scanning and specific genotyping based upon thermal melt profiles
Mol. Genet. Metab.
91
218-227
2007
Homo sapiens (Q8TEY0), Homo sapiens
Manually annotated by BRENDA team
Okano, Y.; Takatori, K.; Kudo, S.; Sakaguchi, T.; Asada, M.; Kajiwara, M.; Yamano, T.
Effects of tetrahydrobiopterin and phenylalanine on in vivo human phenylalanine hydroxylase by phenylalanine breath test
Mol. Genet. Metab.
92
308-314
2007
Homo sapiens
Manually annotated by BRENDA team
Lartey, F.M.; Austic, R.E.
Phenylalanine requirement, imbalance, and dietary excess in one-week-old chicks: growth and phenylalanine hydroxylase activity
Poult. Sci.
87
291-297
2008
Gallus gallus
Manually annotated by BRENDA team
Nascimento, C.; Leandro, J.; Tavares de Almeida, I.; Leandro, P.
Modulation of the activity of newly synthesized human phenylalanine hydroxylase mutant proteins by low-molecular-weight compounds
Protein J.
27
392-400
2008
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Panay, A.J.; Fitzpatrick, P.F.
Kinetic isotope effects on aromatic and benzylic hydroxylation by Chromobacterium violaceum phenylalanine hydroxylase as probes of chemical mechanism and reactivity
Biochemistry
47
11118-11124
2008
Chromobacterium violaceum
Manually annotated by BRENDA team
Boonyapiwat, B.; Forbes, B.; Mitchell, S.; Steventon, G.B.
Phenylalanine 4-monooxygenase and the S-oxidation of S-carboxymethyl-L-cysteine by human cytosolic fractions
Drug Metabol. Drug Interact.
23
261-282
2008
Homo sapiens
Manually annotated by BRENDA team
Lee, Y.W.; Lee, D.H.; Kim, N.D.; Lee, S.T.; Ahn, J.Y.; Choi, T.Y.; Lee, Y.K.; Kim, S.H.; Kim, J.W.; Ki, C.S.
Mutation analysis of PAH gene and characterization of a recurrent deletion mutation in Korean patients with phenylketonuria
Exp. Mol. Med.
40
533-540
2008
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Steventon, G.B.; Mitchell, S.C.
Phenylalanine 4-monooxygenase and the role of endobiotic metabolism enzymes in xenobiotic biotransformation
Expert. Opin. Drug Metab. Toxicol.
5
1213-1221
2009
Rattus norvegicus, Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Brenner, E.; Smagulova, F.; Morozov, I.
Independent origin of rare Y168H mutation of human phenylalanine hydroxylase gene in Russia
Genetika
44
1435-1437
2008
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Pey, A.L.; Martinez, A.
Iron binding effects on the kinetic stability and unfolding energetics of a thermophilic phenylalanine hydroxylase from Chloroflexus aurantiacus
J. Biol. Inorg. Chem.
14
521-531
2009
Chloroflexus aurantiacus
Manually annotated by BRENDA team
Kino, K.; Hara, R.; Nozawa, A.
Enhancement of L-tryptophan 5-hydroxylation activity by structure-based modification of L-phenylalanine 4-hydroxylase from Chromobacterium violaceum
J. Biosci. Bioeng.
108
184-189
2009
Chromobacterium violaceum (P30967), Chromobacterium violaceum, Chromobacterium violaceum NBRC 12614 (P30967)
Manually annotated by BRENDA team
Patel, N.; Iliadou, C.; Boonyapiwat, B.; Barlow, D.; Forbes, B.; Mitchell, S.; Steventon, G.
Enzyme kinetic and molecular modelling studies of sulphur-containing substrates of phenylalanine 4-monooxygenase
J. Enzyme Inhib. Med. Chem.
23
958-963
2008
Homo sapiens, Rattus norvegicus
Manually annotated by BRENDA team
Dobrowolski, S.F.; Borski, K.; Ellingson, C.C.; Koch, R.; Levy, H.L.; Naylor, E.W.
A limited spectrum of phenylalanine hydroxylase mutations is observed in phenylketonuria patients in western Poland and implications for treatment with 6R tetrahydrobiopterin
J. Hum. Genet.
54
335-339
2009
Homo sapiens
Manually annotated by BRENDA team
Dobrowolski, S.F.; Pey, A.L.; Koch, R.; Levy, H.; Ellingson, C.C.; Naylor, E.W.; Martinez, A.
Biochemical characterization of mutant phenylalanine hydroxylase enzymes and correlation with clinical presentation in hyperphenylalaninaemic patients
J. Inherit. Metab. Dis.
32
10-21
2009
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Boonyapiwat, B.; Panaretou, B.; Forbes, B.; Mitchell, S.C.; Steventon, G.B.
Human phenylalanine monooxygenase and thioether metabolism
J. Pharm. Pharmacol.
61
63-67
2009
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Bik-Multanowski, M.; Pietrzyk, J.; Didycz, B.; Szymczakiewicz-Multanowska, A.
Development of a model for assessment of phenylalanine hydroxylase activity in newborns with phenylketonuria receiving tetrahydrobiopterin: A potential for practical implementation
Mol. Genet. Metab.
94
389-390
2008
Homo sapiens (P00439)
Manually annotated by BRENDA team
Steventon, G.B.; Mitchell, S.C.; Perez, B.; Desviat, L.R.; Ugarte, M.
The activity of wild type and mutant phenylalanine hydroxylase with respect to the C-oxidation of phenylalanine and the S-oxidation of S-carboxymethyl-L-cysteine
Mol. Genet. Metab.
96
27-31
2009
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Gunasekera, R.S.; Hyland, K.
In vivo regulation of phenylalanine hydroxylase in the genetic mutant hph-1 mouse model
Mol. Genet. Metab.
98
264-272
2009
Mus musculus
Manually annotated by BRENDA team
Lartey, F.M.; Austic, R.E.
Phenylalanine hydroxylase activity and expression in chicks subjected to phenylalanine imbalance or phenylalanine toxicity
Poult. Sci.
88
774-783
2009
Gallus gallus
Manually annotated by BRENDA team
Stojiljkovic, M.; Perez, B.; Desviat, L.R.; Aguado, C.; Ugarte, M.; Pavlovic, S.
The missense p.S231F phenylalanine hydroxylase gene mutation causes complete loss of enzymatic activity in vitro
Protein J.
28
294-299
2009
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Zhuang, N.; Seo, K.H.; Chen, C.; Kim, H.L.; Park, Y.S.; Lee, K.H.
Purification, crystallization and crystallographic analysis of Dictyostelium discoideum phenylalanine hydroxylase in complex with dihydrobiopterin and FeIII
Acta Crystallogr. Sect. F
66
463-466
2010
Dictyostelium discoideum
Manually annotated by BRENDA team
Flydal, M.I.; Mohn, T.C.; Pey, A.L.; Siltberg-Liberles, J.; Teigen, K.; Martinez, A.
Superstoichiometric binding of L-Phe to phenylalanine hydroxylase from Caenorhabditis elegans: evolutionary implications
Amino Acids
39
1463-1475
2010
Caenorhabditis elegans
Manually annotated by BRENDA team
Li, J.; Ilangovan, U.; Daubner, S.C.; Hinck, A.P.; Fitzpatrick, P.F.
Direct evidence for a phenylalanine site in the regulatory domain of phenylalanine hydroxylase
Arch. Biochem. Biophys.
505
250-255
2011
Rattus norvegicus
Manually annotated by BRENDA team
Li, J.; Dangott, L.J.; Fitzpatrick, P.F.
Regulation of phenylalanine hydroxylase: conformational changes upon phenylalanine binding detected by hydrogen/deuterium exchange and mass spectrometry
Biochemistry
49
3327-3335
2010
Rattus norvegicus (P04176)
Manually annotated by BRENDA team
Leandro, J.; Leandro, P.; Flatmark, T.
Heterotetrameric forms of human phenylalanine hydroxylase: co-expression of wild-type and mutant forms in a bicistronic system
Biochim. Biophys. Acta
1812
602-612
2011
Homo sapiens
Manually annotated by BRENDA team
Gersting, S.W.; Staudigl, M.; Truger, M.S.; Messing, D.D.; Danecka, M.K.; Sommerhoff, C.P.; Kemter, K.F.; Muntau, A.C.
Activation of phenylalanine hydroxylase induces positive cooperativity toward the natural cofactor
J. Biol. Chem.
285
30686-30697
2010
Homo sapiens
Manually annotated by BRENDA team
Boonyapiwat, B.; Mitchell, S.; Steventon, G.
Recombinant heteromeric phenylalanine monooxygenase and the oxygenation of carbon and sulfur substrates
J. Pharm. Pharmacol.
63
558-564
2011
Homo sapiens
Manually annotated by BRENDA team
Lye, L.F.; Kang, S.O.; Nosanchuk, J.D.; Casadevall, A.; Beverley, S.M.
Phenylalanine hydroxylase (PAH) from the lower eukaryote Leishmania major
Mol. Biochem. Parasitol.
175
58-67
2011
Leishmania major (Q6WRI4), Leishmania major
Manually annotated by BRENDA team
Antypa, A.; Rebello, C.; Biernacka, A.; Krajewski, K.; Cassam, J.; Mitchell, S.C.; Steventon, G.B.
Post-translational activation of human phenylalanine 4-monooxygenase from an endobiotic to a xenobiotic enzyme by reactive oxygen and reactive nitrogen species
Xenobiotica
40
319-330
2010
Homo sapiens
Manually annotated by BRENDA team
Jaffe, E.K.; Stith, L.; Lawrence, S.H.; Andrake, M.; Dunbrack, R.L.
A new model for allosteric regulation of phenylalanine hydroxylase: implications for disease and therapeutics
Arch. Biochem. Biophys.
530
73-82
2013
Homo sapiens, Rattus norvegicus (P04176)
Manually annotated by BRENDA team
Roberts, K.M.; Pavon, J.A.; Fitzpatrick, P.F.
Kinetic mechanism of phenylalanine hydroxylase: intrinsic binding and rate constants from single-turnover experiments
Biochemistry
52
1062-1073
2013
Rattus norvegicus
Manually annotated by BRENDA team
Daubner, S.C.; Avila, A.; Bailey, J.O.; Barrera, D.; Bermudez, J.Y.; Giles, D.H.; Khan, C.A.; Shaheen, N.; Thompson, J.W.; Vasquez, J.; Oxley, S.P.; Fitzpatrick, P.F.
Mutagenesis of a specificity-determining residue in tyrosine hydroxylase establishes that the enzyme is a robust phenylalanine hydroxylase but a fragile tyrosine hydroxylase
Biochemistry
52
1446-1455
2013
Homo sapiens (P00439)
Manually annotated by BRENDA team
Ronau, J.A.; Paul, L.N.; Fuchs, J.E.; Corn, I.R.; Wagner, K.T.; Liedl, K.R.; Abu-Omar, M.M.; Das, C.
An additional substrate binding site in a bacterial phenylalanine hydroxylase
Eur. Biophys. J.
42
691-708
2013
Chromobacterium violaceum (P30967), Chromobacterium violaceum, Chromobacterium violaceum ATCC 12472 (P30967)
Manually annotated by BRENDA team
Kim, H.L.; Park, M.B.; Kim, Y.; Yang, Y.G.; Lee, S.W.; Zhuang, N.; Lee, K.H.; Park, Y.S.
Dictyostelium phenylalanine hydroxylase is activated by its substrate phenylalanine
FEBS Lett.
586
3596-3600
2012
Dictyostelium discoideum, Dictyostelium discoideum AX2
Manually annotated by BRENDA team
Flydal, M.I.; Martinez, A.
Phenylalanine hydroxylase: function, structure, and regulation
IUBMB Life
65
341-349
2013
Caenorhabditis elegans, Legionella pneumophila, Homo sapiens (P00439), Homo sapiens, Rattus norvegicus (P04176), Chromobacterium violaceum (P30967), Colwellia psychrerythraea (Q47XN7), Legionella pneumophila 130b
Manually annotated by BRENDA team
Flydal, M.I.; Chatfield, C.H.; Zheng, H.; Gunderson, F.F.; Aubi, O.; Cianciotto, N.P.; Martinez, A.
Phenylalanine hydroxylase from Legionella pneumophila is a thermostable enzyme with a major functional role in pyomelanin synthesis
PLoS ONE
7
e46209
2012
Legionella pneumophila, Legionella pneumophila 130b
Manually annotated by BRENDA team
Carluccio, C.; Fraternali, F.; Salvatore, F.; Fornili, A.; Zagari, A.
Structural features of the regulatory ACT domain of phenylalanine hydroxylase
PLoS ONE
8
e79482
2013
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Shi, Z.; Sellers, J.; Moult, J.
Protein stability and in vivo concentration of missense mutations in phenylalanine hydroxylase
Proteins
80
61-70
2012
Homo sapiens
Manually annotated by BRENDA team
Ronau, J.A.; Paul, L.N.; Fuchs, J.E.; Liedl, K.R.; Abu-Omar, M.M.; Das, C.
A conserved acidic residue in phenylalanine hydroxylase contributes to cofactor affinity and catalysis
Biochemistry
53
6834-6848
2014
Chromobacterium violaceum (P30967), Chromobacterium violaceum
Manually annotated by BRENDA team
Roberts, K.M.; Khan, C.A.; Hinck, C.S.; Fitzpatrick, P.F.
Activation of phenylalanine hydroxylase by phenylalanine does not require binding in the active site
Biochemistry
53
7846-7853
2014
Rattus norvegicus
Manually annotated by BRENDA team
Zhang, S.; Hinck, A.P.; Fitzpatrick, P.F.
The amino acid specificity for activation of phenylalanine hydroxylase matches the specificity for stabilization of regulatory domain dimers
Biochemistry
54
5167-5174
2015
Rattus norvegicus (P04176)
Manually annotated by BRENDA team
Subedi, B.P.; Fitzpatrick, P.F.
Kinetic mechanism and intrinsic rate constants for the reaction of a bacterial phenylalanine hydroxylase
Biochemistry
55
6848-6857
2016
Chromobacterium violaceum (P30967), Chromobacterium violaceum
Manually annotated by BRENDA team
Hayakawa, D.; Yamaotsu, N.; Nakagome, I.; Ozawa, S.I.; Yoshida, T.; Hirono, S.
In silico analyses of the effects of a point mutation and a pharmacological chaperone on the thermal fluctuation of phenylalanine hydroxylase
Biophys. Chem.
228
47-54
2017
Homo sapiens (P00439)
Manually annotated by BRENDA team
Ye, S.; Zhou, L.; Zhou, Z.
Thermal stability improvement for phenylalanine hydroxylase by site-directed mutagenesis
Chin. J. Biotechnol.
32
1243-1254
2016
Chromobacterium violaceum (P30967), Chromobacterium violaceum
Manually annotated by BRENDA team
Ramirez, A.M.; Rodriguez-Lopez, A.; Ardila, A.; Beltran, L.; Patarroyo, C.A.; Melendez, A.D.P.; Sanchez, O.F.; Almeciga-Diaz, C.J.
Production of human recombinant phenylalanine hydroxylase in Lactobacillus plantarum for gastrointestinal delivery
Eur. J. Pharm. Sci.
109
48-55
2017
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Leandro, J.; Stokka, A.J.; Teigen, K.; Andersen, O.A.; Flatmark, T.
Substituting Tyr138 in the active site loop of human phenylalanine hydroxylase affects catalysis and substrate activation
FEBS Open Bio
7
1026-1036
2017
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Leandro, J.; Saraste, J.; Leandro, P.; Flatmark, T.
PKU mutation p.G46S prevents the stereospecific binding of L-phenylalanine to the dimer of human phenylalanine hydroxylase regulatory domain
FEBS open bio
7
195-203
2017
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Pecimonova, M.; Polak, E.; Csicsay, F.; Reblova, K.; Stojiljkovic, M.; Levarski, Z.; Skultety, L.; Kadasi, L.; Soltysova, A.
Functional and structural characterisation of 5 missense mutations of the phenylalanine hydroxylase
Gen. Physiol. Biophys.
36
361-371
2017
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Yu, W.; He, J.; Yang, X.; Zou, H.; Gui, J.; Wang, R.; Yang, L.; Wang, Z.; Lei, Q.
Characterization of phenylalanine hydroxylase gene mutations in phenylketonuria in Xinjiang of China
Int. J. Clin. Exp. Med.
7
4406-4412
2014
Homo sapiens
Manually annotated by BRENDA team
Meisburger, S.P.; Taylor, A.B.; Khan, C.A.; Zhang, S.; Fitzpatrick, P.F.; Ando, N.
Domain movements upon activation of phenylalanine hydroxylase characterized by crystallography and chromatography-coupled small-angle X-ray scattering
J. Am. Chem. Soc.
138
6506-6516
2016
Homo sapiens (P00439), Rattus norvegicus (P04176)
Manually annotated by BRENDA team
Zhang, S.; Fitzpatrick, P.F.
Identification of the allosteric site for phenylalanine in rat phenylalanine hydroxylase
J. Biol. Chem.
291
7418-7425
2016
Rattus norvegicus (P04176)
Manually annotated by BRENDA team
Chadha, N.; Tiwari, A.K.; Kumar, V.; Milton, M.D.; Mishra, A.K.
In silico thermodynamics stability change analysis involved in BH4 responsive mutations in phenylalanine hydroxylase QM/MM and MD simulations analysis
J. Biomol. Struct. Dyn.
33
573-583
2015
Homo sapiens (P00439)
Manually annotated by BRENDA team
Carluccio, C.; Fraternali, F.; Salvatore, F.; Fornili, A.; Zagari, A.
Towards the identification of the allosteric Phe-binding site in phenylalanine hydroxylase
J. Biomol. Struct. Dyn.
34
497-507
2016
Cupriavidus necator, Pseudomonas aeruginosa, Pseudomonas putida, Pseudomonas fluorescens, Xanthomonas campestris, Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Reblova, K.; Kulhanek, P.; Fajkusova, L.
Computational study of missense mutations in phenylalanine hydroxylase
J. Mol. Model.
21
70
2015
Homo sapiens (P00439)
Manually annotated by BRENDA team
Baruteau, J.; Nyabi, O.; Najimi, M.; Fauvart, M.; Sokal, E.
Adult human liver mesenchymal progenitor cells express phenylalanine hydroxylase
J. Pediatr. Endocrinol. Metab.
27
863-868
2014
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Shen, N.; Heintz, C.; Thiel, C.; Okun, J.G.; Hoffmann, G.F.; Blau, N.
Co-expression of phenylalanine hydroxylase variants and effects of interallelic complementation on in vitro enzyme activity and genotype-phenotype correlation
Mol. Genet. Metab.
117
328-335
2016
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Becher, I.; Werner, T.; Doce, C.; Zaal, E.A.; Toegel, I.; Khan, C.A.; Rueger, A.; Muelbaier, M.; Salzer, E.; Berkers, C.R.; Fitzpatrick, P.F.; Bantscheff, M.; Savitski, M.M.
Thermal profiling reveals phenylalanine hydroxylase as an off-target of panobinostat
Nat. Chem. Biol.
12
908-910
2016
Rattus norvegicus
Manually annotated by BRENDA team
Arturo, E.; Gupta, K.; Héroux, A.; Stith, L.; Cross, P.; Parker, E.; Loll, P.; Jaffe, E.
First structure of full-length mammalian phenylalanine hydroxylase reveals the architecture of an autoinhibited tetramer
Proc. Natl. Acad. Sci. USA
113
2394-2399
2016
Rattus norvegicus (P04176)
Manually annotated by BRENDA team
Fuchs, J.; Fuchs, D.; Liedl, K.
Dynamic regulation of phenylalanine hydroxylase
Pteridines
25
33-39
2014
Homo sapiens
-
Manually annotated by BRENDA team
Patel, D.; Kopec, J.; Fitzpatrick, F.; McCorvie, T.J.; Yue, W.W.
Structural basis for ligand-dependent dimerization of phenylalanine hydroxylase regulatory domain
Sci. Rep.
6
23748
2016
Homo sapiens (P00439), Homo sapiens
Manually annotated by BRENDA team
Vandenbossche, E.; Lucas, C.; Mistry, L.; Garfield, E.; Mitchell, S.; Steventon, G.
Phenylalanine monooxygenase and the sulfur oxygenation of S-carboxymethyl-L-cysteine in mice
Xenobiotica
46
379-384
2016
Mus musculus
-
Manually annotated by BRENDA team