


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
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Begoña Bareño
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Kathrin Keim
Members
TECHNICIANS / EARLY STAGE RESEARCHERProtein 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 -
Fernando Lopitz Otsoa
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Nieves Embade
SPECIALIST -
Beatriz González Valle
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Rubén Gil Redondo
POST DOCTORAL RESEARCHER -
David Fernández Ramos
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Andreas Schedlbauer
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Ganeko Bernardo
TECHNICIANS / EARLY STAGE RESEARCHER -
Ángela de Diego Rodríguez
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Ana Laín
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Chiara Bruzzone
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Maider Bizkarguenaga Uribiarte
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Ricardo Diogo Alves Conde
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Riccardo Scarin
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Tania Pereira Ortuzar
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Nekane Merino
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Jon Bilbao García
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Nicanor Zalba Olaizola
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Nerea Adriana Igartua Mendia
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Maria de los Reyes Luque Urbano
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Jon Gil Martinez
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Virginia Gutiérrez de Juan
Members
Latest Publications
Metabolic subtypes of patients with NAFLD exhibit distinctive cardiovascular risk profiles
Martinez-Arranz, I;Bruzzone, C;Noureddin, M;Gil-Redondo, R;Minchole, I;Bizkarguenaga, M;Arretxe, E;Iruarrizaga-Lejarreta, M;Fernandez-Ramos, D;Lopitz-Otsoa, F;Mayo, R;Embade, N;Newberry, E;Mittendorf, ...
HEPATOLOGY
2022-03-17
Exploration of Human Serum Lipoprotein Supramolecular Phospholipids Using Statistical Heterospectroscopy in n-Dimensions (SHY-n): Identification of Potential Cardiovascular Risk Biomarkers Related to SARS-CoV-2 Infection
Masuda, R;Lodge, S;Whiley, L;Gray, N;Lawler, N;Nitschke, P;Bong, SH;Kimhofer, T;Loo, RL;Boughton, B;Zeng, AX;Hall, D;Schaefer, H;Spraul, M;Dwivedi, G;Yeap, BB;Diercks, T;Bernardo-Seisdedos, ...
ANALYTICAL CHEMISTRY
2022-03-15
Depletion of mitochondrial methionine adenosyltransferase alpha 1 triggers mitochondrial dysfunction in alcohol-associated liver disease
Barbier-Torres, L;Murray, B;Yang, JW;Wang, JH;Matsuda, M;Robinson, A;Binek, A;Fan, W;Fernandez-Ramos, D;Lopitz-Otsoa, F;Luque-Urbano, M;Millet, O;Mavila, N;Peng, H;Ramani, K;Gottlieb, R;Sun, ...
NATURE COMMUNICATIONS
2022-01-28
J-Edited DIffusional Proton Nuclear Magnetic Resonance Spectroscopic Measurement of Glycoprotein and Supramolecular Phospholipid Biomarkers of Inflammation in Human Serum
Nitschke, P;Lodge, S;Kimhofer, T;Masuda, R;Bong, SH;Hall, D;Schafer, H;Spraul, M;Pompe, N;Diercks, T;Bernardo-Seisdedos, G;Mato, JM;Millet, O;Susic, D;Henry, A;El-Omar, EM;Holmes, E;Lindon, ...
ANALYTICAL CHEMISTRY
2022-01-05
Conformation-sensitive antibody reveals an altered cytosolic PAS/CNBh assembly during hERG channel gating
Harley, CA;Bernardo-Seisdedos, G;Stevens-Sostre, WA;Jones, DK;Azevedo, MM;Sampaio, P;Lorga-Gomes, M;Trudeau, MC;Millet, O;Robertson, GA;Morais-Cabral, JH
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
2021-11-01
Uneven metabolic and lipidomic profiles in recovered COVID-19 patients as investigated by plasma NMR metabolomics
Bizkarguenaga, M;Bruzzone, C;Gil-Redondo, R;SanJuan, I;Martin-Ruiz, I;Barriales, D;Palacios, A;Pasco, ST;Gonzalez-Valle, B;Lain, A;Herrera, L;Azkarate, A;Vesga, MA;Eguizabal, C;Anguita, J;Embade, ...
NMR IN BIOMEDICINE
2021-10-27
Metabolomics and lipidomics in NAFLD: biomarkers and non-invasive diagnostic tests
Masoodi, M;Gastaldelli, A;Hyotylainen, T;Arretxe, E;Alonso, C;Gaggini, M;Brosnan, J;Anstee, QM;Millet, O;Ortiz, P;Mato, JM;Dufour, JF;Oresic, M
NATURE REVIEWS GASTROENTEROLOGY & HEPATOLOGY
2021-09-10
O-GlcNAcylated p53 in the liver modulates hepatic glucose production
Gonzalez-Rellan, MJ;Fondevila, MF;Fernandez, U;Rodriguez, A;Varela-Rey, M;Veyrat-Durebex, C;Seoane, S;Bernardo, G;Lopitz-Otsoa, F;Fernandez-Ramos, D;Bilbao, J;Iglesias, C;Novoa, E;Ameneiro, ...
NATURE COMMUNICATIONS
2021-08-20
Integrative Modeling of Plasma Metabolic and Lipoprotein Biomarkers of SARS-CoV-2 Infection in Spanish and Australian COVID-19 Patient Cohorts
Masuda, R;Lodge, S;Nitschke, P;Spraul, M;Schaefer, H;Bong, SH;Kimhofer, T;Hall, D;Loo, RL;Bizkarguenaga, M;Bruzzone, C;Gil-Redondo, R;Embade, N;Mato, JM;Holmes, E;Wist, J;Millet, O;Nicholson, ...
JOURNAL OF PROTEOME RESEARCH
2021-08-06
A molecular signature for the metabolic syndrome by urine metabolomics
Bruzzone, C;Gil-Redondo, R;Seco, M;Barragan, R;de la Cruz, L;Cannet, C;Schafer, H;Fang, F;Diercks, T;Bizkarguenaga, M;Gonzalez-Valle, B;Lain, A;Sanz-Parra, A;Coltell, O;de Letona, AL;Spraul, ...
CARDIOVASCULAR DIABETOLOGY
2021-07-28