Jon Bilbao García
jbilbao
Jon Bilbao García
EARLY STAGE RESEARCHER
Precision Medicine and Metabolism Lab & Metabolomics Platform
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.
Latest Publications

Targeting Hepatic Glutaminase 1 Ameliorates Non-alcoholic Steatohepatitis by Restoring Very-Low-Density Lipoprotein Triglyceride Assembly

Simon, J;Nunez-Garcia, M;Fernandez-Tussy, P;Barbier-Torres, L;Fernandez-Ramos, D;Gomez-Santos, B;Buque, X;Lopitz-Otsoa, F;Goikoetxea-Usandizaga, N;Serrano-Macia, M;Rodriguez-Agudo, R;Bizkarguenaga, ...

CELL METABOLISM

2020-03-03

Early treatment efficacy of S-adenosylmethionine in patients with intrahepatic cholestasis: A systematic review

Noureddin, M;Sander-Struckmeier, S;Mato, JM

WORLD JOURNAL OF HEPATOLOGY

2020-02-27

Methionine Adenosyltransferase alpha 1 Is Targeted to the Mitochondrial Matrix and Interacts with Cytochrome P450 2E1 to Lower Its Expression

Murray, B;Peng, H;Barbier-Torres, L;Robinson, AE;Li, TWH;Fan, W;Tomasi, ML;Gottlieb, RA;Van Eyk, J;Lu, ZM;Mart?nez-Chantar, ML;Liangpunsakul, S;Skill, NJ;Mato, JM;Lu, SC

Hepatology (Baltimore, Md.)

2019-12-01

miR-873-5p targets mitochondrial GNMT-Complex II interface contributing to non-alcoholic fatty liver disease

Fernandez-Tussy, P;Fernandez-Ramos, D;Lopitz-Otsoa, F;Simon, J;Barbier-Torres, L;Gomez-Santos, B;Nunez-Garcia, M;Azkargorta, M;Gutierrez-de Juan, V;Serrano-Macia, M;Rodriguez-Agudo, R;Iruzubieta, ...

MOLECULAR METABOLISM

2019-11-01

Methionine adenosyltransferases in liver cancer

Murray, B;Barbier-Torres, L;Fan, W;Mato, JM;Lu, SC

WORLD JOURNAL OF GASTROENTEROLOGY

2019-08-21

Biomarkers and subtypes of deranged lipid metabolism in non-alcoholic fatty liver disease

Mato, JM;Alonso, C;Noureddin, M;Lu, SC

WORLD JOURNAL OF GASTROENTEROLOGY

2019-06-28

Control and regulation of S-Adenosylmethionine biosynthesis by the regulatory beta subunit and quinolone-based compounds

Panmanee, J;Bradley-Clarke, J;Mato, JM;O'Neill, PM;Antonyuk, SV;Hasnain, SS

FEBS JOURNAL

2019-06-01

Metabolomics discloses potential biomarkers to predict the acute HVPG response to propranolol in patients with cirrhosis

Reverter, E;Lozano, JJ;Alonso, C;Berzigotti, A;Seijo, S;Turon, F;Baiges, A;Martinez-Chantar, ML;Mato, JM;Martinez-Arranz, I;La Mura, V;Hernandez-Gea, V;Bosch, J;Garcia-Pagan, JC

LIVER INTERNATIONAL

2019-04-01

NEDDylation inhibition as a new potential therapy of nonalcoholic fatty liver disease

Serrano-Macia, M;Azkargorta, M;Simon, J;Otsoa, FL;Delgado, TC;Ramos, DF;de Juan, VG;Varela-Rey, M;Goikoetxea, N;Tussy, PF;Apichueta, P;de Urturi, DS;Buque, X;Iruzubieta, P;Crespo, J;Lu, SC;Mato, ...

JOURNAL OF HEPATOLOGY

2019-04-01

Mitochondrial GNMT-complex II is recovered by miR-873-5p targeting in NAFLD

Ramos, DF;Tussy, PF;Otsoa, FL;Simon, J;Torres, LB;Santos, BG;Nunez, M;Azkargorta, M;de Juan, VG;Serrano-Macia, M;Agudo, RR;Champagne, DG;Iruzubieta, P;Anguita, J;Rincon, M;Elortza, F;Anita, ...

JOURNAL OF HEPATOLOGY

2019-04-01

Latest Publications

Patients with Cholangiocarcinoma Present Specific RNA Profiles in Serum and Urine Extracellular Vesicles Mirroring the Tumor Expression: Novel Liquid Biopsy Biomarkers for Disease Diagnosis

Lapitz, A;Arbelaiz, A;O'Rourke, CJ;Lavin, JL;La Casta, A;Ibarra, C;Jimeno, JP;Santos-Laso, A;Izquierdo-Sanchez, L;Krawczyk, M;Perugorria, MJ;Jimenez-Aguero, R;Sanchez-Campos, A;Riano, I;Gonzalez, ...

CELLS

2020-03-14

Targeting Hepatic Glutaminase 1 Ameliorates Non-alcoholic Steatohepatitis by Restoring Very-Low-Density Lipoprotein Triglyceride Assembly

Simon, J;Nunez-Garcia, M;Fernandez-Tussy, P;Barbier-Torres, L;Fernandez-Ramos, D;Gomez-Santos, B;Buque, X;Lopitz-Otsoa, F;Goikoetxea-Usandizaga, N;Serrano-Macia, M;Rodriguez-Agudo, R;Bizkarguenaga, ...

CELL METABOLISM

2020-03-03

Considerations towards a roadmap for collection, handling and storage of blood extracellular vesicles

Clayton, A;Boilard, E;Buzas, EI;Cheng, L;Falcon-Perez, JM;Gardiner, C;Gustafson, D;Gualerzi, A;Hendrix, A;Hoffmann, A;Jones, J;Lasser, C;Lawson, C;Lenassi, M;Nazarenko, I;O'Driscoll, L;Pink, ...

JOURNAL OF EXTRACELLULAR VESICLES

2019-12-01

Biological membranes in EV biogenesis, stability, uptake, and cargo transfer: an ISEV position paper arising from the ISEV membranes and EVs workshop

Russell, AE;Sneider, A;Witwer, KW;Bergese, P;Bhattacharyya, SN;Cocks, A;Cocucci, E;Erdbrugger, U;Falcon-Perez, JM;Freeman, DW;Gallagher, TM;Hu, SS;Huang, YY;Jay, SM;Kano, S;Lavieu, G;Leszczynska, ...

JOURNAL OF EXTRACELLULAR VESICLES

2019-12-01

Metabolic Nano-Machines: Extracellular Vesicles Containing Active Enzymes and Their Contribution to Liver Diseases

Angulo, MA (Azparren Angulo, Maria)[ 1 ] ; Royo, F (Royo, Felix)¡; Falcón-Pérez, JM (Falcon-Perez, Juan M.)

CURRENT PATHOBIOLOGY REPORTS

2019-12-01

Metabolomics Applied to the Study of Extracellular Vesicles

Williams, C;Palviainen, M;Reichardt, NC;Siljander, PRM;Falcon-Perez, JM

Metabolites

2019-11-12

EV-associated miRNAs from pleural lavage as potential diagnostic biomarkers in lung cancer

Roman-Canal, B;Moiola, CP;Gatius, S;Bonnin, S;Ruiz-Miro, M;Gonzalez, E;Ojanguren, A;Recuero, JL;Gil-Moreno, A;Falcon-Perez, JM;Ponomarenko, J;Porcel, JM;Matias-Guiu, X;Colas, E

SCIENTIFIC REPORTS

2019-10-21

Glycoprofile Analysis of an Intact Glycoprotein As Inferred by NMR Spectroscopy

Unione, L;Lenza, MP;Arda, A;Urquiza, P;Lain, A;Falcon-Perez, JM;Jimenez-Barbero, J;Millet, O

ACS CENTRAL SCIENCE

2019-09-25

Hereditary tyrosinemia type I-associated mutations in fumarylacetoacetate hydrolase reduce the enzyme stability and increase its aggregation rate

Macias, I;Lain, A;Bernardo-Seisdedos, G;Gil, D;Gonzalez, E;Falcon-Perez, JM;Millet, O

JOURNAL OF BIOLOGICAL CHEMISTRY

2019-08-30

Assessing the role of surface glycans of extracellular vesicles on cellular uptake

Williams, C;Pazos, R;Royo, F;Gonzalez, E;Roura-Ferrer, M;Martinez, A;Gamiz, J;Reichardt, NC;Falcon-Perez, JM

SCIENTIFIC REPORTS

2019-08-15