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A. L. Pey · SciRah

Researcher profile · Semantic Scholar

A. L. Pey

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A. L. Pey — researcher profile on SciRah

h-index: 33
Works: 125
Citations: 3,419

Most-cited papers

Primary hyperoxalurias: disorders of glyoxylate detoxification.
E. Salido, A. L. Pey, R. Rodriguez, V. Lorenzo
2012Biochimica et Biophysica Acta168 citations Open access
Semantic Scholar
Human cystathionine β-synthase (CBS) contains two classes of binding sites for S-adenosylmethionine (SAM): complex regulation of CBS activity and stability by SAM.
A. L. Pey, T. Majtan, J. Sanchez-Ruiz, J. Kraus
2013Biochemical Journal102 citations
Semantic Scholar
NQO1: A target for the treatment of cancer and neurological diseases, and a model to understand loss of function disease mechanisms.
S. Beaver, Noel Mesa-Torres, A. L. Pey, D. Timson
2019Biochimica et Biophysica Acta - Proteins and Proteomics85 citations Open access
Semantic Scholar
NAD(P)H quinone oxidoreductase (NQO1): an enzyme which needs just enough mobility, in just the right places
A. L. Pey, C. Megarity, D. Timson
2018Bioscience Reports69 citations Open access
Semantic Scholar
FAD binding overcomes defects in activity and stability displayed by cancer-associated variants of human NQO1.
A. L. Pey, C. Megarity, D. Timson
2014Biochimica et Biophysica Acta64 citations Open access
Semantic Scholar
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
A. L. Pey, E. Salido, J. Sanchez-Ruiz
2011Amino Acids64 citations
Semantic Scholar
Structural Basis of the Oncogenic Interaction of Phosphatase PRL-1 with the Magnesium Transporter CNNM2*♦
Paula Giménez-Mascarell, I. Oyenarte, S. Hardy, T. Breiderhoff +11 more
2016Journal of Biological Chemistry60 citations Open access
Semantic Scholar
The Role of Protein Denaturation Energetics and Molecular Chaperones in the Aggregation and Mistargeting of Mutants Causing Primary Hyperoxaluria Type I
Noel Mesa-Torres, Israel Fabelo-Rosa, D. Riverol, C. Yunta +3 more
2013PLoS ONE60 citations Open access
Semantic Scholar
BANK1 interacts with TRAF6 and MyD88 in innate immune signaling in B cells
I. Georg, A. Díaz-Barreiro, M. Morell, A. L. Pey +1 more
2019Cellular & Molecular Immunology53 citations Open access
Semantic Scholar
Intrinsically disordered chromatin protein NUPR1 binds to the C-terminal region of Polycomb RING1B
P. Santofimia-Castaño, B. Rizzuti, A. L. Pey, Philippe Soubeyran +4 more
2017Proceedings of the National Academy of Sciences of the United States of America53 citations Open access
Semantic Scholar
Domain Organization, Catalysis and Regulation of Eukaryotic Cystathionine Beta-Synthases
T. Majtan, A. L. Pey, Roberto Fernández, J. A. Fernández +2 more
2014PLoS ONE53 citations Open access
Semantic Scholar
Protein Homeostasis Defects of Alanine-Glyoxylate Aminotransferase: New Therapeutic Strategies in Primary Hyperoxaluria Type I
A. L. Pey, A. Albert, E. Salido
2013BioMed Research International51 citations Open access
Semantic Scholar
Molecular basis of classic galactosemia from the structure of human galactose 1-phosphate uridylyltransferase
T. McCorvie, J. Kopeć, A. L. Pey, F. Fitzpatrick +4 more
2016Human Molecular Genetics49 citations Open access
Semantic Scholar
Conformational dynamics is key to understanding loss-of-function of NQO1 cancer-associated polymorphisms and its correction by pharmacological ligands
Encarnación Medina-Carmona, R. Palomino-Morales, Julian E. Fuchs, Esperanza Padín-González +4 more
2016Scientific Reports46 citations Open access
Semantic Scholar
Insight into the specificity and severity of pathogenic mechanisms associated with missense mutations through experimental and structural perturbation analyses
Encarnación Medina-Carmona, I. Betancor-Fernández, Jaime Santos, Noel Mesa-Torres +8 more
2018Human Molecular Genetics41 citations Open access
Semantic Scholar
Site-to-site interdomain communication may mediate different loss-of-function mechanisms in a cancer-associated NQO1 polymorphism
Encarnación Medina-Carmona, J. Neira, E. Salido, Julian E. Fuchs +3 more
2017Scientific Reports40 citations Open access
Semantic Scholar
Second Generation of Mannich Base-Type Derivatives with in Vivo Activity against Trypanosoma cruzi.
Rubén Martín-Escolano, Elsa Moreno-Viguri, Mery Santivañez-Veliz, Á. Martín-Montes +8 more
2018Journal of Medicinal Chemistry35 citations Open access
Semantic Scholar
Enhanced vulnerability of human proteins towards disease-associated inactivation through divergent evolution
Encarnación Medina-Carmona, Julian E. Fuchs, J. Gavira, Noel Mesa-Torres +6 more
2017Human Molecular Genetics35 citations Open access
Semantic Scholar
Potential Pharmacological Chaperones for Cystathionine Beta-Synthase-Deficient Homocystinuria.
T. Majtan, A. L. Pey, Paula Giménez-Mascarell, L. Martínez-Cruz +3 more
2018Handbook of Experimental Pharmacology34 citations Open access
Semantic Scholar
Targeting Cystathionine Beta-Synthase Misfolding in Homocystinuria by Small Ligands: State of the Art and Future Directions.
T. Majtan, A. L. Pey, J. Ereño-Orbea, L. Martínez-Cruz +1 more
2016Current Drug Targets34 citations Open access
Semantic Scholar
The consensus-based approach for gene/enzyme replacement therapies and crystallization strategies: the case of human alanine-glyoxylate aminotransferase.
Noel Mesa-Torres, C. Yunta, Israel Fabelo-Rosa, J. González-Rubio +4 more
2014Biochemical Journal32 citations Open access
Semantic Scholar
The Catalytic Cycle of the Antioxidant and Cancer-Associated Human NQO1 Enzyme: Hydride Transfer, Conformational Dynamics and Functional Cooperativity
Ernesto Anoz-Carbonell, D. Timson, A. L. Pey, M. Medina
2020Antioxidants30 citations Open access
Semantic Scholar
Natural Small Molecules as Stabilizers and Activators of Cancer-Associated NQO1 Polymorphisms.
A. L. Pey, C. Megarity, Encarnación Medina-Carmona, D. Timson
2016Current Drug Targets29 citations Open access
Semantic Scholar
The metastability of human UDP-galactose 4'-epimerase (GALE) is increased by variants associated with type III galactosemia but decreased by substrate and cofactor binding.
A. L. Pey, Esperanza Padín-González, Noel Mesa-Torres, D. Timson
2014Archives of Biochemistry and Biophysics29 citations Open access
Semantic Scholar
Conformational properties of nine purified cystathionine beta-synthase mutants
A. Hnízda, T. Majtan, Lu Liu, A. L. Pey +4 more
2012Biochemistry29 citations Open access
Semantic Scholar
Thermodynamics of cooperative binding of FAD to human NQO1: Implications to understanding cofactor-dependent function and stability of the flavoproteome.
Rafael Claveria-Gimeno, A. Velázquez-Campoy, A. L. Pey
2017Archives of Biochemistry and Biophysics28 citations
Semantic Scholar
Kinetic stability of cystathionine beta-synthase can be modulated by structural analogs of S-adenosylmethionine: Potential approach to pharmacological chaperone therapy for homocystinuria.
T. Majtan, A. L. Pey, J. Kraus
2016Biochimie28 citations
Semantic Scholar
The lower limits for protein stability and foldability in primary hyperoxaluria type I.
Noel Mesa-Torres, E. Salido, A. L. Pey
2014Biochimica et Biophysica Acta28 citations
Semantic Scholar
A mechanism for cancer‐associated inactivation of NQO1 due to P187S and its reactivation by the consensus mutation H80R
I. G. Muñoz, B. Morel, Encarnación Medina-Carmona, A. L. Pey
2017FEBS Letters27 citations Open access
Semantic Scholar
Structural and energetic basis of protein kinetic destabilization in human phosphoglycerate kinase 1 deficiency.
A. L. Pey, Noel Mesa-Torres, L. Chiarelli, G. Valentini
2013Biochemistry27 citations
Semantic Scholar
Structural basis of the pleiotropic and specific phenotypic consequences of missense mutations in the multifunctional NAD(P)H:quinone oxidoreductase 1 and their pharmacological rescue
Juan Luis Pacheco-García, Ernesto Anoz-Carbonell, P. Vaňková, Adithi Kannan +7 more
2021Redox Biology26 citations Open access
Semantic Scholar
Divergence in enzyme regulation between Caenorhabditis elegans and human tyrosine hydroxylase, the key enzyme in the synthesis of dopamine.
A. Calvo, A. L. Pey, A. Miranda-Vizuete, A. Døskeland +1 more
2011Biochemical Journal26 citations
Semantic Scholar
A Dynamic Core in Human NQO1 Controls the Functional and Stability Effects of Ligand Binding and Their Communication across the Enzyme Dimer
P. Vaňková, E. Salido, D. Timson, P. Man +1 more
2019Biomolecules25 citations Open access
Semantic Scholar
Protein Stability, Folding and Misfolding in Human PGK1 Deficiency
G. Valentini, M. Maggi, A. L. Pey
2013Biomolecules25 citations Open access
Semantic Scholar
Phosphorylation compromises FAD binding and intracellular stability of wild-type and cancer-associated NQO1: Insights into flavo-proteome stability.
Encarnación Medina-Carmona, B. Rizzuti, Rubén Martín-Escolano, Juan Luis Pacheco-García +4 more
2019International Journal of Biological Macromolecules23 citations Open access
Semantic Scholar
Protein homeostasis disorders of key enzymes of amino acids metabolism: mutation-induced protein kinetic destabilization and new therapeutic strategies
A. L. Pey
2013Amino Acids22 citations
Semantic Scholar
Biophysical and functional perturbation analyses at cancer-associated P187 and K240 sites of the multifunctional NADP(H):quinone oxidoreductase 1.
A. L. Pey
2018International Journal of Biological Macromolecules21 citations
Semantic Scholar
Insights into human phosphoglycerate kinase 1 deficiency as a conformational disease from biochemical, biophysical, and in vitro expression analyses
A. L. Pey, M. Maggi, G. Valentini
2014Journal of Inherited Metabolic Disease21 citations
Semantic Scholar
Systemic Alanine Glyoxylate Aminotransferase mRNA Improves Glyoxylate Metabolism in a Mouse Model of Primary Hyperoxaluria Type 1
A. Kukreja, M. Lasaro, Christian W. Cobaugh, C. Forbes +7 more
2019Nucleic Acid Therapeutics19 citations
Semantic Scholar
Galactokinase promiscuity: a question of flexibility?
M. McAuley, H. Kristiansson, Mei-Lan Huang, A. L. Pey +1 more
2016Biochemical Society Transactions19 citations Open access
Semantic Scholar
Structural and functional insights on the roles of molecular chaperones in the mistargeting and aggregation phenotypes associated with primary hyperoxaluria type I.
J. Fernández-Higuero, I. Betancor-Fernández, Noel Mesa-Torres, A. Muga +2 more
2019Advances in Protein Chemistry and Structural Biology17 citations
Semantic Scholar
Molecular Recognition of PTS-1 Cargo Proteins by Pex5p: Implications for Protein Mistargeting in Primary Hyperoxaluria
Noel Mesa-Torres, N. Tomić, A. Albert, E. Salido +1 more
2015Biomolecules17 citations Open access
Semantic Scholar
New salicylic acid derivatives, double inhibitors of glycolate oxidase and lactate dehydrogenase, as effective agents decreasing oxalate production.
Maria Dolores Moya-Garzon, Barbara Rodriguez-Rodriguez, C. Martín-Higueras, Francisco Franco-Montalbán +5 more
2022European journal of medicinal chemistry16 citations Open access
Semantic Scholar
Thermodynamic versus kinetic basis for the high conformational stability of nanobodies for therapeutic applications
Atanasio Gómez-Mulas, Mario Cano-Muñoz, Eduardo Salido Ruiz, A. L. Pey
2024bioRxiv15 citations Open access
Semantic Scholar
Galactosemia: Towards Pharmacological Chaperones
Samantha Banford, T. McCorvie, A. L. Pey, D. Timson
2021Journal of Personalized Medicine15 citations Open access
Semantic Scholar
Warfarin increases thermal resistance of albumin through stabilization of the protein lobe that includes its binding site.
B. Rizzuti, R. Bartucci, A. L. Pey, R. Guzzi
2019Archives of Biochemistry and Biophysics15 citations Open access
Semantic Scholar
Evolutionary Divergent Suppressor Mutations in Conformational Diseases
Noel Mesa-Torres, I. Betancor-Fernández, Elisa Oppici, B. Cellini +2 more
2018Genes15 citations Open access
Semantic Scholar
Natural (and Unnatural) Small Molecules as Pharmacological Chaperones and Inhibitors in Cancer.
I. Betancor-Fernández, D. Timson, E. Salido, A. L. Pey
2018Handbook of Experimental Pharmacology15 citations
Semantic Scholar
Oligomeric status of human cystathionine beta‐synthase modulates AdoMet binding
A. L. Pey, L. Martínez-Cruz, J. Kraus, T. Majtan
2016FEBS Letters15 citations Open access
Semantic Scholar
Superstoichiometric binding of L-Phe to phenylalanine hydroxylase from Caenorhabditis elegans: evolutionary implications
M. Flydal, Tonje C. Mohn, A. L. Pey, J. Siltberg-Liberles +2 more
2010Amino Acids15 citations
Semantic Scholar

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Noel Mesa-Torres×14
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Encarnación Medina-Carmona×8
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