Crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals the structural basis for phenylketonuria
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Direct evidence for a phenylalanine site in the regulatory domain of phenylalanine hydroxylaseRegulation of phenylalanine hydroxylase: conformational changes upon phenylalanine binding detected by hydrogen/deuterium exchange and mass spectrometryMechanism of aromatic amino acid hydroxylationProtein design: toward functional metalloenzymesStructure of phenylalanine hydroxylase from Colwellia psychrerythraea 34H, a monomeric cold active enzyme with local flexibility around the active site and high overall stabilityMechanism of Inhibition of Novel Tryptophan Hydroxylase Inhibitors Revealed by Co-crystal Structures and Kinetic AnalysisStructural and mechanistic basis of the interaction between a pharmacological chaperone and human phenylalanine hydroxylaseStructural and thermodynamic insight into phenylalanine hydroxylase from the human pathogenLegionella pneumophilaStructure of tetrameric human phenylalanine hydroxylase and its implications for phenylketonuriaMissense mutations in the N-terminal domain of human phenylalanine hydroxylase interfere with binding of regulatory phenylalanineThe accessibility of iron at the active site of recombinant human phenylalanine hydroxylase to water as studied by 1H NMR paramagnetic relaxation. Effect of L-Phe and comparison with the rat enzyme.Characterization of a stable form of tryptophan hydroxylase from the human parasite Schistosoma mansoni.Essential role of the N-terminal autoregulatory sequence in the regulation of phenylalanine hydroxylase.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 cooperativPurification, crystallization and crystallographic analysis of Dictyostelium discoideum phenylalanine hydroxylase in complex with dihydrobiopterin and FeIII.Twisting of the DNA-binding surface by a beta-strand-bearing proline modulates DNA gyrase activity.Mutagenesis of the regulatory domain of phenylalanine hydroxylase.Phenylalanine hydroxylase from Legionella pneumophila is a thermostable enzyme with a major functional role in pyomelanin synthesis.Dynamic regulation of phenylalanine hydroxylase by simulated redox manipulationPhenylalanine hydroxylase misfolding and pharmacological chaperones.First structure of full-length mammalian phenylalanine hydroxylase reveals the architecture of an autoinhibited tetramerLoss of function in phenylketonuria is caused by impaired molecular motions and conformational instabilityCorrection of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations.EPR of Mononuclear Non-Heme Iron Proteins.Tetrahydrobiopterin, its mode of action on phenylalanine hydroxylase, and importance of genotypes for pharmacological therapy of phenylketonuria.An ongoing debate over phenylalanine hydroxylase deficiency in phenylketonuria.Phenylketonuria: genotype-phenotype correlations based on expression analysis of structural and functional mutations in PAH.Computational study of missense mutations in phenylalanine hydroxylase.Stabilization of tryptophan hydroxylase 2 by l-phenylalanine-induced dimerization.Kinetic mechanism of phenylalanine hydroxylase: intrinsic binding and rate constants from single-turnover experiments.Predicted effects of missense mutations on native-state stability account for phenotypic outcome in phenylketonuria, a paradigm of misfolding diseases.Demonstration of a peroxide shunt in the tetrahydropterin-dependent aromatic amino acid monooxygenases.Substrate-induced conformational transition in human phenylalanine hydroxylase as studied by surface plasmon resonance analyses: the effect of terminal deletions, substrate analogues and phosphorylation.A flexible loop in tyrosine hydroxylase controls coupling of amino acid hydroxylation to tetrahydropterin oxidation.Identification by hydrogen/deuterium exchange of structural changes in tyrosine hydroxylase associated with regulation.Kinetic isotope effects on aromatic and benzylic hydroxylation by Chromobacterium violaceum phenylalanine hydroxylase as probes of chemical mechanism and reactivity.The effect of substrate, dihydrobiopterin, and dopamine on the EPR spectroscopic properties and the midpoint potential of the catalytic iron in recombinant human phenylalanine hydroxylase.Assessing the relative importance of the biophysical properties of amino acid substitutions associated with human genetic disease.Phosphorylation and mutations of Ser(16) in human phenylalanine hydroxylase. Kinetic and structural effects.Probing cofactor specificity in phenylalanine hydroxylase by molecular dynamics simulations.
P2860
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P2860
Crystal structure of the catalytic domain of human phenylalanine hydroxylase reveals the structural basis for phenylketonuria
description
1997 nî lūn-bûn
@nan
1997 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1997 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
1997年の論文
@ja
1997年学术文章
@wuu
1997年学术文章
@zh-cn
1997年学术文章
@zh-hans
1997年学术文章
@zh-my
1997年学术文章
@zh-sg
1997年學術文章
@yue
name
Crystal structure of the catal ...... ural basis for phenylketonuria
@ast
Crystal structure of the catal ...... ural basis for phenylketonuria
@en
Crystal structure of the catal ...... ural basis for phenylketonuria
@nl
type
label
Crystal structure of the catal ...... ural basis for phenylketonuria
@ast
Crystal structure of the catal ...... ural basis for phenylketonuria
@en
Crystal structure of the catal ...... ural basis for phenylketonuria
@nl
prefLabel
Crystal structure of the catal ...... ural basis for phenylketonuria
@ast
Crystal structure of the catal ...... ural basis for phenylketonuria
@en
Crystal structure of the catal ...... ural basis for phenylketonuria
@nl
P2093
P2860
P3181
P356
P1476
Crystal structure of the catal ...... ural basis for phenylketonuria
@en
P2093
P2860
P304
P3181
P356
10.1038/NSB1297-995
P577
1997-12-01T00:00:00Z