IMI2 Programme
0000-0003-1264-3153
0000-0001-8748-4105José M Mato is founder and General Director of the Centers bioGUNE (Bilbao) and biomaGUNE (San Sebastián), and Research Professor of the Spanish National Research Council (CSIC), Spain. A graduate in Biochemistry at the Complutense University of Madrid, Professor Mato received a PhD degree from Leiden University and was awarded the CJ Kok prize for his thesis. He was a postdoctoral fellow at the Biozentrum of the University of Basel and the National Institutes of Health, and a faculty member at the Jiménez Díaz Foundation in Madrid before been named Research Professor at the CSIC. He has been Professor of the Faculty of Medicine of the University of Navarra and Visiting Professor at the University of Pennsylvania and Thomas Jeff erson University. From 1992 to 1996 Professor Mato was President of the CSIC and in 2004 was awarded the Spanish National Research Prize in Medicine.
Latest Publications
2022
2021
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Natxo Reguera Pascual
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Begoña Bareño
Members
TECHNICIANS / DOCTORAL CANDIDATESProtein stability (thermodynamic and kinetic) drives the biophysical properties of the polypeptide chain (protein folding) and the protein's concentration in the cellular environment (protein homeostasis). It is the result of a delicate balance between inter- and intramolecular interactions, which can be easily altered by mutations and/or upon changes in the composition of the surrounding media. In this context, NMR spectroscopy offers a plethora of suitable experiments to investigate protein stability. In our laboratory we are currently interested in the following topics:
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Pharmacological chaperones. Rare diseases (~7000 identified to date) are an area of significant medical need affecting an estimated 350 million people worldwide, with ~95% having no currently approved drug treatment. They are often produced by inherited mutations affecting the activity of a protein and It is becoming increasily clear that, most frequently, a mutation destabilizes the protein/enzyme, ultimately affecting its intracellular homeostasis. In this context, pharmacological chaperones (small molecules which bind to the protein, restoring stability and activity without affecting its function) can be applied to many diseases. In our laboratory we are investigating new methods (NMR, biophysical and biochemical) for the discovery and characterization of pharmacological chaperones against a set of diseases: congenital eryhtropoietic porphyria, tyrosianemia.
- Environmental modulation of enzyme stability. The high catalytic efficiency and the exquisite enantioselectivity of an enzyme has been employed in some industrial processes to upgrade their properties in order to make them more environmentally-friendly. However, large scale industrial implementation of biotechnological reactions is often limited by the marginal stability of the enzyme in the reactor conditions. In our laboratory we employ NMR and circular dichroism to investigate the effect of external crowding agents to improve the activity and stability of several enzymes. Specifically, we are investigating the mechanism for protein haloadaptation by a combined use of site directed mutagenesis and high-resolution NMR spectroscopy.
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Principal Investigator
Óscar Millet
IKERBASQUE RESEARCH FELLOW -
Gabriel Ortega Quintanilla
Ikerbasque Research Fellow RESEARCH ASSISTANT -
Nieves Embade
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Fernando Lopitz Otsoa
SPECIALIST -
Rubén Gil Redondo
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Beatriz González Valle
POSTDOCTORAL RESEARCHER -
Andreas Schedlbauer
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David Fernández Ramos
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Alain Ibañez de Opakua Lopez de Abetxuko
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Ganeko Bernardo
TECHNICIANS / DOCTORAL CANDIDATES -
Maider Bizkarguenaga Uribiarte
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Cristina Garrido
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Ana Laín
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Tania Pereira Ortuzar
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Jon Gil Martinez
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Pablo Herrero Alfonso
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Sara Martín Ramos
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Uxue Hermoso Martínez
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Laura Gálvez Larrosa
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Karen Salua Alarcón Morales
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Alba Pejenaute Pejenaute
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Riccardo Scarin
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Maria de los Reyes Luque Urbano
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Ángela de Diego Rodríguez
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Margarita Gómez Galán
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Virginia Gutiérrez de Juan
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Ricardo Diogo Alves Conde
Members
Latest Publications
Hepatocyte-specific O-GlcNAc transferase downregulation ameliorates nonalcoholic steatohepatitis by improving mitochondrial function
Gonzalez-Rellan, MJ; Parracho, T; Heras, V; Rodriguez, A; Fondevila, MF; Novoa, E; Lima, N; Varela-Rey, M; Senra, A; Chantada-Vazquez, MDP; Ameneiro, C; Bernardo, G; Fernandez-Ramos, D; Lopitz-Otsoa, ...
MOLECULAR METABOLISM
2023-09-01
Hepatic levels of S-adenosylmethionine regulate the adaptive response to fasting
Capelo-Diz, A; Lachiondo-Ortega, S; Fernández-Ramos, D; Cañas-Martín, J; Goikoetxea-Usandizaga, N; Serrano-Maciá, M; González-Rellan, MJ; Mosca, L; Blazquez-Vicens, J; Tinahones-Ruano, A; Fondevila, ...
CELL METABOLISM
2023-08-08
Serum and Urine Metabolomic Profiling of Newly Diagnosed Treatment-Naive Inflammatory Bowel Disease Patients
Aldars-García, L; Gil-Redondo, R; Embade, N; Riestra, S; Rivero, M; Gutiérrez, A; Rodríguez-Lago, I; Fernández-Salazar, L; Ceballos, D; Benitez, JM; Aguas, M; Baston-Rey, I; Bermejo, F; Casanova, ...
INFLAMMATORY BOWEL DISEASES
2023-08-03
Integrative Plasma Metabolic and Lipidomic Modelling of SARS-CoV-2 Infection in Relation to Clinical Severity and Early Mortality Prediction
Lodge, S; Lawler, NG; Gray, N; Masuda, R; Nitschke, P; Whiley, L; Bong, SH; Yeap, BB; Dwivedi, G; Spraul, M; Schaefer, H; Gil-Redondo, R; Embade, N; Millet, O; Holmes, E; Wist, J; Nicholson, ...
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
2023-07-01
Urinary Metabolomic Profile of Youth at Risk of Chronic Kidney Disease in Nicaragua
Hall, SM; Raines, NH; Ramirez-Rubio, O; Amador, JJ; López-Pilarte, D; OCallaghan-Gordo, C; Gil-Redondo, R; Embade, N; Millet, O; Peng, XJ; Vences, S; Keogh, SA; Delgado, IS; Friedman, DJ; Brooks, ...
KIDNEY360
2023-07-01
Metabolic Features of Increased Gut Permeability, Inflammation, and Altered Energy Metabolism Distinguish Agricultural Workers at Risk for Mesoamerican Nephropathy
Raines, NH; Leone, DA; OCallaghan-Gordo, C; Ramirez-Rubio, O; Amador, JJ; Pilarte, DL; Delgado, IS; Leibler, JH; Embade, N; Gil-Redondo, R; Bruzzone, C; Bizkarguenaga, M; Scammell, MK; Parikh, ...
METABOLITES
2023-03-01
Redox regulation of KV7 channels through EF3 hand of calmodulin
Nunez, E; Jones, F; Muguruza-Montero, A; Urrutia, J; Aguado, A; Malo, C; Bernardo-Seisdedos, G; Domene, C; Millet, O; Gamper, N; Villarroel, A; Colecraft, HM;
ELIFE
2023-02-20
Hyperphosphorylation of hepatic proteome characterizes nonalcoholic fatty liver disease in S-adenosylmethionine deficiency
Robinson, AE; Binek, A; Ramani, K; Sundararaman, N; Barbier-Torres, L; Murray, B; Venkatraman, V; Kreimer, S; Mc Ardle, A; Noureddin, M; Fernandez-Ramos, D; Lopitz-Otsoa, F; de Juan, VG; Millet, ...
iScience
2023-02-17
Metabolomics as a powerful tool for diagnostic, pronostic and drug intervention analysis in COVID-19
Bruzzone, C; Conde, R; Embade, N; Mato, JM; Millet, O;
FRONTIERS IN MOLECULAR BIOSCIENCES
2023-02-15
Protoporphyrin IX Binds to Iron(II)-Loaded and to Zinc-Loaded Human Frataxin
Bernardo-Seisdedos, G; Schedlbauer, A; Pereira-Ortuzar, T; Mato, JM; Millet, O;
LIFE-BASEL
2023-01-01
NMR Investigation of Protein-Carbohydrate Interactions: The Recognition of Glycans by Galectins Engineered with Fluorotryptophan Residues
Lete, MG; Franconetti, A; Bertuzzi, S; Delgado, S; Azkargorta, M; Elortza, F; Millet, O; Jiménez-Osés, G; Arda, A; Jiménez-Barbero, J;
CHEMISTRY-A EUROPEAN JOURNAL
2022-12-12
An NMR-Based Model to Investigate the Metabolic Phenoreversion of COVID-19 Patients throughout a Longitudinal Study
Gil-Redondo, R; Conde, R; Bizkarguenaga, M; Bruzzone, C; Laín, A; González-Valle, B; Iriberri, M; Ramos-Acosta, C; Anguita, E; Lariz, JIA; Yandiola, PPE; Moran, MA; Jiménez-Mercado, ME; Egia-Mendikute, ...
METABOLITES
2022-12-01
A flow cytometry-based neutralization assay for simultaneous evaluation of blocking antibodies against SARS-CoV-2 variants
Egia-Mendikute, L; Bosch, A; Prieto-Fernández, E; Vila-Vecilla, L; Zanetti, SR; Lee, SY; Jiménez-Lasheras, B; del Río, AG; Antoñana-Vildosola, A; de Blas, A; Velasco-Beltrán, P; Serrano-Maciá, ...
FRONTIERS IN IMMUNOLOGY
2022-11-24
A glutamine-based single α-helix scaffold to target globular proteins
Escobedo, A; Piccirillo, J; Aranda, J; Diercks, T; Mateos, B; Garcia-Cabau, C; Sánchez-Navarro, M; Topal, B; Biesaga, M; Staby, L; Kragelund, BB; García, J; Millet, O; Orozco, M; Coles, M; Crehuet, ...
NATURE COMMUNICATIONS
2022-11-18
The use of pharmacological chaperones in rare diseases caused by reduced protein stability
Gil-Martínez, J; Bernardo-Seisdedos, G; Mato, JM; Millet, O;
PROTEOMICS
2022-10-19
ALAD Inhibition by Porphobilinogen Rationalizes the Accumulation of ?-Aminolevulinate in Acute Porphyrias br
San Juan, I; Pereira-Ortuzar, T; Cendoya, X; Laín, A; To-Figueras, J; Mateos, B; Planes, FJ; Bernardo-Seisdedos, G; Mato, JM; Millet, O;
BIOCHEMISTRY
2022-10-14
Fine-tuning of the Hsc70-based Human Protein Disaggregase Machinery by the Distinctive C-terminal Extension of Apg2
Cabrera, Y; Bernardo-Seisdedos, G; Dublang, L; Albesa-Jove, D; Orozco, N; Viguera, AR; Millet, O; Muga, A; Moro, F;
JOURNAL OF MOLECULAR BIOLOGY
2022-10-06
Direct low field J-edited diffusional proton NMR spectroscopic measurement of COVID-19 inflammatory biomarkers in human serum
Nitschke, P; Lodge, S; Hall, D; Schaefer, H; Spraul, M; Embade, N; Millet, O; Holmes, E; Wist, J; Nicholson, JK;
ANALYST
2022-08-16
Inhibition of carnitine palmitoyltransferase 1A in hepatic stellate cells protects against fibrosis
Fondevila, MF; Fernandez, U; Heras, V; Parracho, T; Gonzalez-Rellan, MJ; Novoa, E; Porteiro, B; Alonso, C; Mayo, R; Lima, ND; Iglesias, C; Filliol, AA; Senra, A; Delgado, TC; Woodhoo, A; Herrero, ...
JOURNAL OF HEPATOLOGY
2022-06-15
Assessing the Mobility of Severe Acute Respiratory Syndrome Coronavirus-2 Spike Protein Glycans by Structural and Computational Methods
Stagnoli, S; Peccati, F; Connell, SR; Martinez-Castillo, A; Charro, D; Millet, O; Bruzzone, C; Palazon, A; Arda, A; Jimenez-Barbero, J; Ereno-Orbea, J; Abrescia, NGA; Jimenez-Oses, G;
FRONTIERS IN MICROBIOLOGY
2022-04-15