Ganeko Bernardo
gbernardo
Ganeko Bernardo
POSTDOCTORAL RESEARCHER
Precision Medicine and Metabolism Lab
Email: gbernardo.atlas@cicbiogune.es
Phone: 4452
Address: Bizkaia Science and Technology Park, building 800, Derio (Bizkaia)

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

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

Impaired Function of Solute Carrier Family 19 Leads to Low Folate Levels and Lipid Droplet Accumulation in Hepatocytes

Cano, A;Vazquez-Chantada, M;Conde-Vancells, J;Gonzalez-Lahera, A;Mosen-Ansorena, D;Blanco, FJ;Clement, K;Aron-Wisnewsky, J;Tran, A;Gual, P;Garcia-Monzon, C;Caballeria, J;Castro, A;Martinez-Chantar, ...

BIOMEDICINES

2023-02-01

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

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

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

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

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

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