José M Mato
jmmato
José M Mato
GENERAL DIRECTOR
IMI2 Programme
IMI2 Programme
General Director Office &
Precision Medicine and Metabolism Lab
Address: Bizkaia Science and Technology Park, building 801A, Derio (Bizkaia)
omillet
Óscar Millet
Group Leader
Precision Medicine and Metabolism Lab
Address: Bizkaia Science and Technology Park, building 800, Derio (Bizkaia)

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

Protein 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:

  • 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.

Protocolos de Histología

Latest Publications

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

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

2023-12-25

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

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

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; Fernández-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