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Reference on EC 1.14.16.1 - phenylalanine 4-monooxygenase and Organism(s) Homo sapiens and UniProt Accession P00439

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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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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