Angel Luis Pey
Angel Luis Pey
Associate Professor, Department of Physical Chemistry, Universidad de Granada (Spain)
Dirección de correo verificada de ugr.es
TítuloCitado porAño
Correction of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations
H Erlandsen, AL Pey, A Gámez, B Pérez, LR Desviat, C Aguado, R Koch, ...
Proceedings of the National Academy of Sciences 101 (48), 16903-16908, 2004
1792004
Predicted effects of missense mutations on native-state stability account for phenotypic outcome in phenylketonuria, a paradigm of misfolding diseases
AL Pey, F Stricher, L Serrano, A Martinez
The American Journal of Human Genetics 81 (5), 1006-1024, 2007
1702007
Identification of pharmacological chaperones as potential therapeutic agents to treat phenylketonuria
AL Pey, M Ying, N Cremades, A Velazquez-Campoy, T Scherer, B Thöny, ...
The Journal of clinical investigation 118 (8), 2858-2867, 2008
1482008
Phenylketonuria: Genotype–phenotype correlations based on expression analysis of structural and functional mutations in PAH
AL Pey, LR Desviat, A Gámez, M Ugarte, B Pérez
Human mutation 21 (4), 370-378, 2003
1412003
Mechanisms underlying responsiveness to tetrahydrobiopterin in mild phenylketonuria mutations
AL Pey, B Pérez, LR Desviat, MA Martínez, C Aguado, H Erlandsen, ...
Human mutation 24 (5), 388-399, 2004
1302004
Primary hyperoxalurias: disorders of glyoxylate detoxification
E Salido, AL Pey, R Rodriguez, V Lorenzo
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1822 (9 …, 2012
1042012
Effects of prolonged stanozolol treatment on antioxidant enzyme activities, oxidative stress markers, and heat shock protein HSP72 levels in rat liver
A Pey, A Saborido, I Blázquez, J Delgado, A Megı́as
The Journal of steroid biochemistry and molecular biology 87 (4-5), 269-277, 2003
722003
Structure of phenylalanine hydroxylase from Colwellia psychrerythraea 34H, a monomeric cold active enzyme with local flexibility around the active site and high overall stability
HKS Leiros, AL Pey, M Innselset, E Moe, I Leiros, IH Steen, A Martinez
Journal of Biological Chemistry 282 (30), 21973-21986, 2007
652007
Human cystathionine beta-synthase (CBS) contains two classes of binding sites for S-adenosyl-L-methionine (SAM): complex regulation of CBS activity and stability by SAM.
AL Pey, T Majtan, JM Sanchez-Ruiz, JP Kraus
Biochemical Journal 449 (1), 109-121, 2013
642013
Role of low native state kinetic stability and interaction of partially unfolded states with molecular chaperones in the mitochondrial protein mistargeting associated with …
AL Pey, E Salido, JM Sanchez-Ruiz
Amino acids 41 (5), 1233-1245, 2011
532011
The role of protein denaturation energetics and molecular chaperones in the aggregation and mistargeting of mutants causing primary hyperoxaluria type I
N Mesa-Torres, I Fabelo-Rosa, D Riverol, C Yunta, A Albert, E Salido, ...
PLoS One 8 (8), e71963, 2013
432013
The activity of wild-type and mutant phenylalanine hydroxylase and its regulation by phenylalanine and tetrahydrobiopterin at physiological and pathological concentrations: an …
AL Pey, A Martinez
Molecular genetics and metabolism 86, 43-53, 2005
432005
Thermodynamic characterization of the binding of tetrahydropterins to phenylalanine hydroxylase
AL Pey, M Thórólfsson, K Teigen, M Ugarte, A Martínez
Journal of the American Chemical Society 126 (42), 13670-13678, 2004
412004
Specific interaction of the diastereomers 7 (R)-and 7 (S)-tetrahydrobiopterin with phenylalanine hydroxylase: implications for understanding primapterinuria and vitiligo
AL Pey, A Martinez, R Charubala, DJ Maitland, K Teigen, A Calvo, ...
The FASEB journal 20 (12), 2130-2132, 2006
402006
Engineering proteins with tunable thermodynamic and kinetic stabilities
AL Pey, D Rodriguez‐Larrea, S Bomke, S Dammers, R Godoy‐Ruiz, ...
Proteins: Structure, Function, and Bioinformatics 71 (1), 165-174, 2008
392008
Rescuing proteins of low kinetic stability by chaperones and natural ligands: phenylketonuria, a case study
A Martinez, AC Calvo, K Teigen, AL Pey
Progress in molecular biology and translational science 83, 89-134, 2008
392008
Biochemical characterization of mutant phenylalanine hydroxylase enzymes and correlation with clinical presentation in hyperphenylalaninaemic patients
SF Dobrowolski, AL Pey, R Koch, H Levy, CC Ellingson, EW Naylor, ...
Journal of inherited metabolic disease 32 (1), 10, 2009
362009
FAD binding overcomes defects in activity and stability displayed by cancer-associated variants of human NQO1
AL Pey, CF Megarity, DJ Timson
Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1842 (11 …, 2014
352014
Effect of pharmacological chaperones on brain tyrosine hydroxylase and tryptophan hydroxylase 2
AC Calvo, T Scherer, AL Pey, M Ying, I Winge, J McKinney, J Haavik, ...
Journal of neurochemistry 114 (3), 853-863, 2010
312010
Molecular basis of classic galactosemia from the structure of human galactose 1-phosphate uridylyltransferase
TJ McCorvie, J Kopec, AL Pey, F Fitzpatrick, D Patel, R Chalk, L Shrestha, ...
Human molecular genetics 25 (11), 2234-2244, 2016
302016
El sistema no puede realizar la operación en estos momentos. Inténtalo de nuevo más tarde.
Artículos 1–20