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
Principal Investigator
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.

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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

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;Jimenez-Oses, G;Arda, A;Jimenez-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;Lain, A;Gonzalez-Valle, B;Iriberri, M;Ramos-Acosta, C;Anguita, E;Lariz, JIA;Yandiola, PPE;Moran, MA;Jimenez-Mercado, ME;Egia-Mendikute, L;Seco, ...

METABOLITES

2022-12-01

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

2022-11-30

A flow cytometry-based neutralization assay for simultaneous evaluation of blocking antibodies against SARS-CoV-2 variants

Egia-Mendikute, L;Bosch, A;Prieto-Fernandez, E;Vila-Vecilla, L;Zanetti, SR;Lee, SY;Jimenez-Lasheras, B;del Rio, AG;Antonana-Vildosola, A;de Blas, A;Velasco-Beltran, P;Serrano-Macia, M;Iruzubieta, ...

FRONTIERS IN IMMUNOLOGY

2022-11-24

A glutamine-based single alpha-helix scaffold to target globular proteins

Escobedo, A;Piccirillo, J;Aranda, J;Diercks, T;Mateos, B;Garcia-Cabau, C;Sanchez-Navarro, M;Topal, B;Biesaga, M;Staby, L;Kragelund, BB;Garcia, J;Millet, O;Orozco, M;Coles, M;Crehuet, R;Salvatella, ...

NATURE COMMUNICATIONS

2022-11-18

The use of pharmacological chaperones in rare diseases caused by reduced protein stability

Gil-Martinez, 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;Lain, A;To-Figueras, J;Mateos, B;Planes, FJ;Bernardo-Seisdedos, G;Mato, JM;Millet, O

BIOCHEMISTRY

2022-10-14

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, L;Serra, ...

JOURNAL OF HEPATOLOGY

2022-07-01

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