| 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 | 230 | 2007 |
| 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 | 226 | 2004 |
| 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 | 203 | 2012 |
| 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 | 199 | 2008 |
| 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 | 165 | 2004 |
| 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 | 164 | 2003 |
| 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 | 101 | 2003 |
| 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 | 100 | 2013 |
| NQO1: A target for the treatment of cancer and neurological diseases, and a model to understand loss of function disease mechanisms SK Beaver, N Mesa-Torres, AL Pey, DJ Timson Biochimica et Biophysica Acta (BBA)-Proteins and Proteomics 1867 (7-8), 663-676, 2019 | 92 | 2019 |
| 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 | 88 | 2007 |
| NAD (P) H quinone oxidoreductase (NQO1): an enzyme which needs just enough mobility, in just the right places AL Pey, CF Megarity, DJ Timson Bioscience reports 39 (1), BSR20180459, 2019 | 83 | 2019 |
| 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 | 72 | 2014 |
| 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, 1233-1245, 2011 | 69 | 2011 |
| Structural basis of the oncogenic interaction of phosphatase PRL-1 with the magnesium transporter CNNM2 P Giménez-Mascarell, I Oyenarte, S Hardy, T Breiderhoff, M Stuiver, ... Journal of Biological Chemistry 292 (3), 786-801, 2017 | 66 | 2017 |
| 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 | 65 | 2016 |
| 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 | 60 | 2013 |
| Intrinsically disordered chromatin protein NUPR1 binds to the C-terminal region of Polycomb RING1B P Santofimia-Castaño, B Rizzuti, ÁL Pey, P Soubeyran, M Vidal, R Urrutia, ... Proceedings of the National Academy of Sciences 114 (31), E6332-E6341, 2017 | 57 | 2017 |
| Domain organization, catalysis and regulation of eukaryotic cystathionine beta-synthases T Majtan, AL Pey, R Fernández, JA Fernández, LA Martínez-Cruz, ... PloS one 9 (8), e105290, 2014 | 56 | 2014 |
| 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 | 56 | 2008 |
| 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 | 55 | 2005 |
Aurora MartinezProfessor ved University of BergenBestätigte E-Mail-Adresse bei uib.no
