BIOMOLECULAS
BIOMOLECULAS
University of Bergen
Bergen, NoruegaPublicaciones en colaboración con investigadores/as de University of Bergen (24)
2012
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The Peripheral Binding of 14-3-3γ to Membranes Involves Isoform-Specific Histidine Residues
PLoS ONE, Vol. 7, Núm. 11
2011
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Divergence in enzyme regulation between Caenorhabditis elegans and human tyrosine hydroxylase, the key enzyme in the synthesis of dopamine
Biochemical Journal, Vol. 434, Núm. 1, pp. 133-141
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The regulatory subunit of PKA-I remains partially structured and undergoes β-aggregation upon thermal denaturation
PLoS ONE, Vol. 6, Núm. 3
2010
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Effect of pharmacological chaperones on brain tyrosine hydroxylase and tryptophan hydroxylase 2
Journal of Neurochemistry, Vol. 114, Núm. 3, pp. 853-863
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Phenylalanine hydroxylase expression in primary rat hepatocytes is modulated by oxygen concentration
Molecular Genetics and Metabolism, Vol. 101, Núm. 2-3, pp. 279-281
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Superstoichiometric binding of L-Phe to phenylalanine hydroxylase from Caenorhabditis elegans: Evolutionary implications
Amino Acids, Vol. 39, Núm. 5, pp. 1463-1475
2009
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Biochemical characterization of mutant phenylalanine hydroxylase enzymes and correlation with clinical presentation in hyperphenylalaninaemic patients
Journal of Inherited Metabolic Disease, Vol. 32, Núm. 1, pp. 10-21
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Iron binding effects on the kinetic stability and unfolding energetics of a thermophilic phenylalanine hydroxylase from Chloroflexus aurantiacus
Journal of Biological Inorganic Chemistry, Vol. 14, Núm. 4, pp. 521-531
2008
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Anabolic function of phenylalanine hydroxylase in Caenorhabditis elegans
FASEB Journal, Vol. 22, Núm. 8, pp. 3046-3058
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Chapter 3 Rescuing Proteins of Low Kinetic Stability by Chaperones and Natural Ligands. Phenylketonuria, a Case Study
Progress in Nucleic Acid Research and Molecular Biology, Vol. 83, pp. 89-134
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Engineering proteins with tunable thermodynamic and kinetic stabilities
Proteins: Structure, Function and Genetics, Vol. 71, Núm. 1, pp. 165-174
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Identification of pharmacological chaperones as potential therapeutic agents to treat phenylketonuria
Journal of Clinical Investigation, Vol. 118, Núm. 8, pp. 2858-2867
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Large-scale modulation of thermodynamic protein folding barriers linked to electrostatics
Proceedings of the National Academy of Sciences of the United States of America, Vol. 105, Núm. 25, pp. 8625-8630
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Thermoplasma acidophilum Cdc6 protein stimulates MCM helicase activity by regulating its ATPase activity.
Nucleic acids research, Vol. 36, Núm. 17, pp. 5602-5609
2007
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Predicted effects of missense mutations on native-state stability account for phenotypic outcome in phenylketonuria, a paradigm of misfolding diseases
American Journal of Human Genetics, Vol. 81, Núm. 5, pp. 1006-1024
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Structure of phenylalanine hydroxylase from Colwellia psychrerythraea 34H, a monomeric cold active enzyme with local flexibility around the active site and high overall stability
Journal of Biological Chemistry, Vol. 282, Núm. 30, pp. 21973-21986
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Tetrahydrobiopterin for patients with phenylketonuria
Lancet
2006
2005
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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
Molecular Genetics and Metabolism, Vol. 86, Núm. SUPPL., pp. 43-53
2004
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Correction of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations
Proceedings of the National Academy of Sciences of the United States of America, Vol. 101, Núm. 48, pp. 16903-16908