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Human cystathionine β-synthase (CBS) contains two classes of binding sites for S-adenosylmethionine (SAM): complex regulation of CBS activity and stability by SAMPrimary hyperoxalurias: disorders of glyoxylate detoxificationThe Role of Protein Denaturation Energetics and Molecular Chaperones in the Aggregation and Mistargeting of Mutants Causing Primary Hyperoxaluria Type IThe consensus-based approach for gene/enzyme replacement therapies and crystallization strategies: the case of human alanine-glyoxylate aminotransferaseDomain organization, catalysis and regulation of eukaryotic cystathionine beta-synthasesProtein homeostasis disorders of key enzymes of amino acids metabolism: mutation-induced protein kinetic destabilization and new therapeutic strategies.Molecular basis of classic galactosemia from the structure of human galactose 1-phosphate uridylyltransferase.Structural Basis of the Oncogenic Interaction of Phosphatase PRL-1 with the Magnesium Transporter CNNM2.The regulatory subunit of PKA-I remains partially structured and undergoes β-aggregation upon thermal denaturation.The role of surface electrostatics on the stability, function and regulation of human cystathionine β-synthase, a complex multidomain and oligomeric proteinThe chondroitin sulfate/dermatan sulfate 4-O-endosulfatase from marine bacterium Vibrio sp FC509 is a dimeric species: Biophysical characterization of an endosulfatase.Identification of pharmacological chaperones as potential therapeutic agents to treat phenylketonuria.Thermoplasma acidophilum Cdc6 protein stimulates MCM helicase activity by regulating its ATPase activityCorrection of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations.Protein homeostasis defects of alanine-glyoxylate aminotransferase: new therapeutic strategies in primary hyperoxaluria type I.Protein Stability, Folding and Misfolding in Human PGK1 Deficiency.Natural Small Molecules as Stabilizers and Activators of Cancer-Associated NQO1 Polymorphisms.Galactokinase promiscuity: a question of flexibility?Role of low native state kinetic stability and interaction of partially unfolded states with molecular chaperones in the mitochondrial protein mistargeting associated with primary hyperoxaluria.Divergence in enzyme regulation between Caenorhabditis elegans and human tyrosine hydroxylase, the key enzyme in the synthesis of dopamine.Phenylketonuria: genotype-phenotype correlations based on expression analysis of structural and functional mutations in PAH.Site-to-site interdomain communication may mediate different loss-of-function mechanisms in a cancer-associated NQO1 polymorphism.Predicted effects of missense mutations on native-state stability account for phenotypic outcome in phenylketonuria, a paradigm of misfolding diseases.Molecular recognition of PTS-1 cargo proteins by Pex5p: implications for protein mistargeting in primary hyperoxaluria.pH-dependent relationship between thermodynamic and kinetic stability in the denaturation of human phosphoglycerate kinase 1.Correction to Conformational Properties of Nine Purified Cystathionine β-Synthase Mutants.Conformational dynamics is key to understanding loss-of-function of NQO1 cancer-associated polymorphisms and its correction by pharmacological ligands.Erratum: Conformational dynamics is key to understanding loss-of-function of NQO1 cancer-associated polymorphisms and its correction by pharmacological ligands.Phenylalanine hydroxylase expression in primary rat hepatocytes is modulated by oxygen concentration.Iron binding effects on the kinetic stability and unfolding energetics of a thermophilic phenylalanine hydroxylase from Chloroflexus aurantiacus.Effect of pharmacological chaperones on brain tyrosine hydroxylase and tryptophan hydroxylase 2.Superstoichiometric binding of L-Phe to phenylalanine hydroxylase from Caenorhabditis elegans: evolutionary implications.FAD binding overcomes defects in activity and stability displayed by cancer-associated variants of human NQO1.Structural and energetic basis of protein kinetic destabilization in human phosphoglycerate kinase 1 deficiency.Effects of prolonged stanozolol treatment on antioxidant enzyme activities, oxidative stress markers, and heat shock protein HSP72 levels in rat liver.Mechanisms underlying responsiveness to tetrahydrobiopterin in mild phenylketonuria mutations.Thermodynamic characterization of the binding of tetrahydropterins to phenylalanine hydroxylase.Rescuing proteins of low kinetic stability by chaperones and natural ligands phenylketonuria, a case study.Biochemical characterization of mutant phenylalanine hydroxylase enzymes and correlation with clinical presentation in hyperphenylalaninaemic patients.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.
P50
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P50
description
hulumtues
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researcher
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wetenschapper
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հետազոտող
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name
Angel L Pey
@nl
Angel L Pey
@sl
Angel L. Pey
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Angel L. Pey
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type
label
Angel L Pey
@nl
Angel L Pey
@sl
Angel L. Pey
@en
Angel L. Pey
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prefLabel
Angel L Pey
@nl
Angel L Pey
@sl
Angel L. Pey
@en
Angel L. Pey
@es
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P106
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P31
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0000-0001-7706-3243