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José 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
2023
2022
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Begoña Bareño
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Natxo Reguera Pascual
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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Group Leader
Óscar Millet
Emerging Scientist -
Gabriel Ortega Quintanilla
Ikerbasque Research Fellow - Ramón y Cajal (RyC) Programme RESEARCH ASSISTANT -
Fernando Lopitz Otsoa
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Nieves Embade
SPECIALIST -
Beatriz González Valle
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Rubén Gil Redondo
POSTDOCTORAL RESEARCHER -
Ganeko Bernardo
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David Fernández Ramos
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Alain Ibañez de Opakua Lopez de Abetxuko
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Andreas Schedlbauer
TECHNICIANS / DOCTORAL CANDIDATES -
Virginia Gutiérrez de Juan
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Ricardo Diogo Alves Conde
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Maria de los Reyes Luque Urbano
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Karen Salua Alarcón Morales
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Riccardo Scarin
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Margarita Gómez Galán
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Jon Gil Martinez
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Pablo Herrero Alfonso
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Alba Pejenaute Pejenaute
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Laura Gálvez Larrosa
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Tania Pereira Ortuzar
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Sara Martín Ramos
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Uxue Hermoso Martínez
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Cristina Garrido
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Maider Bizkarguenaga Uribiarte
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Ángela de Diego Rodríguez
Members
Latest Publications
Pseudophosphorylation of single residues of the J-domain of DNAJA2 regulates the holding/folding balance of the Hsc70 system.
Velasco-Carneros, Lorea; Bernardo-Seisdedos, Ganeko; Marechal, Jean-Didier; Millet, Oscar; Moro, Fernando; Muga, Arturo;
Protein science : a publication of the Protein Society
2024-08-01
Seroprevalence of adeno-associated virus types 1, 2, 3, 4, 5, 6, 8, and 9 in a Basque cohort of healthy donors.
Navarro-Oliveros, Miguel; Vidaurrazaga, Ander; Soares Guerra, Gabriel; Castellana, Donatello; Embade, Nieves; Millet, Oscar; Marigorta, Urko M; Abrescia, Nicola G A;
Scientific reports
2024-07-10
Preovulatory follicular fluid secretome added to in vitro maturation medium influences the metabolism of equine cumulus-oocyte complexes
Luis-Calero, M; Ortiz-Rodríguez, JM; Fernández-Hernández, P; Muñoz-García, CC; Pericuesta, E; Gutiérrez-Adán, A; Marinaro, F; Embade, N; Conde, R; Bizkarguenaga, M; Millet, O; González-Fernández, ...
BMC VETERINARY RESEARCH
2024-06-25
Electrostatics introduce a trade-off between mesophilic stability and adaptation in halophilic proteins
Herrero-Alfonso, P; Pejenaute, A; Millet, O; Ortega-Quintanilla, G;
PROTEIN SCIENCE
2024-06-01
Congenital erythropoietic porphyria
To-Figueras, J; Erwin, AL; Aguilera, P; Millet, O; Desnick, RJ;
LIVER INTERNATIONAL
2024-05-08
Stratification of Sepsis Patients on Admission into the Intensive Care Unit According to Differential Plasma Metabolic Phenotypes
Lodge, S; Litton, E; Gray, N; Ryan, M; Millet, O; Fear, M; Raby, E; Currie, A; Wood, F; Holmes, E; Wist, J; Nicholson, JK;
JOURNAL OF PROTEOME RESEARCH
2024-03-21
Cross-Validation of Metabolic Phenotypes in SARS-CoV-2 Infected Subpopulations Using Targeted Liquid Chromatography-Mass Spectrometry (LC-MS)
Whiley, L; Lawler, NG; Zeng, AX; Lee, A; Chin, ST; Bizkarguenaga, M; Bruzzone, C; Embade, N; Wist, J; Holmes, E; Millet, O; Nicholson, JK; Gray, N;
JOURNAL OF PROTEOME RESEARCH
2024-03-14
Characterization of preovulatory follicular fluid secretome and its effects on equine oocytes during in vitro maturation
Luis-Calero, M; Marinaro, F; Fernández-Hernández, P; Ortiz-Rodríguez, JM; Casado, JG; Pericuesta, E; Gutiérrez-Adán, A; González, E; Azkargorta, M; Conde, R; Bizkarguenaga, M; Embade, N; Elortza, ...
RESEARCH IN VETERINARY SCIENCE
2024-03-11
Impaired Hepatic Very Low-Density Lipoprotein Secretion Promotes Tumorigenesis and Is Accelerated with Fabp1 Deletion.
Newberry, Elizabeth P; Molitor, Elizabeth A; Liu, Allen; Chong, Kamyar; Liu, Xiuli; Alonso, Cristina; Mato, Jose M; Davidson, Nicholas O;
The American journal of pathology
2024-02-28
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
2024-02-01
Quantitative Analysis of the Human Semen Phosphorometabolome by 31P-NMR
Serrano, R; Martin-Hidalgo, D; Bilbao, J; Bernardo-Seisdedos, G; Millet, O; Garcia-Marin, LJ; Bragado, MJ;
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
2024-02-01
Effect of age and dietary habits on Red Blood Cell membrane fatty acids in a Southern Europe population (Basque Country)
Marrugat, G; Cano, A; Amézaga, J; Arranz, S; Embade, N; Millet, O; Ferreri, C; Tueros, I;
PROSTAGLANDINS LEUKOTRIENES AND ESSENTIAL FATTY ACIDS
2024-01-01
Serum identification of at-risk MASH: The metabolomics-advanced steatohepatitis fibrosis score (MASEF)
Noureddin, M; Truong, E; Mayo, R; Martínez-Arranz, I; Banales, JM; Mincholé, I; Arrese, M; Cusi, K; Arias-Loste, MT; Bruha, R; Romero-Gómez, M; Iruzubieta, P; Aller, R; Ampuero, J; Calleja, ...
HEPATOLOGY
2024-01-01
S-Adenosylmethionine Negatively Regulates the Mitochondrial Respiratory Chain Repressor MCJ in the Liver
Barbier-Torres, L; Chhimwal, J; Kim, SY; Ramani, K; Robinson, A; Yang, HP; Van Eyk, J; Liangpunsakul, S; Seki, E; Mato, JM; Lu, SC;
INTERNATIONAL JOURNAL OF BIOLOGICAL SCIENCES
2024-01-01
Hepatic prohibitin 1 and methionine adenosyltransferase a1 defend against primary and secondary liver cancer metastasis
Fan, W; Cao, DY; Yang, B; Wang, JH; Li, XM; Kitka, D; Li, TWH; You, SY; Shiao, S; Gangi, A; Posadas, E; Di Vizio, D; Tomasi, ML; Seki, E; Mato, JM; Yang, HP; Lu, SC;
JOURNAL OF HEPATOLOGY
2023-12-10
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
Performance of non-invasive tests and histology for the prediction of clinical outcomes in patients with non-alcoholic fatty liver disease: an individual participant data meta-analysis
Mózes, FE; Lee, JA; Vali, Y; Alzoubi, O; Staufer, K; Trauner, M; Paternostro, R; Stauber, RE; Holleboom, AG; van Dijk, AM; Mak, AL; Boursier, J; Loup, MD; Shima, T; Bugianesi, E; Gaia, S; Armandi, ...
Lancet Gastroenterology & Hepatology
2023-08-01
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