He holds degrees in Chemistry (Univ. Ramon Llull,1994) and Chemical Engineering (IQS, 1995). After obtaining a Ph D in Organic Chemistry (University of Barcelona, 1999) he joined the group of Lewis Kay in Toronto for a post-doctoral stay (University of Toronto, 2000-2004). He was then recipient of a Ramon y Cajal reincorporation contract at the Parc Cientific de Barcelona (2004-2006) and he currently is group leader at the CIC bioGUNE. His research line focuses of the use of nuclear magnetic resonance (NMR) to the study of biologically relevant proteins and enzymes, paying special attention to the delicate balance existing between protein stability and dynamics. Such knowledge is applied for the development of new compounds with therapeutic activity, specifically in the field of rare diseases. He have published more than 55 papers with a total number of citations (1998-2015) of 2050 and an h-index of 17. He was the Spanish delegate for the trans domain of the COST program (2009-2014). He was awarded the prize of the Real Sociedad Española de Química (2004) and the Spanish NMR group prize (2005). He currently is the president of the Spanish NMR group.
Side chain to main chain hydrogen bonds stabilize a polyglutamine helix in a transcription factor
Escobedo, A;Topal, B;Kunze, MBA;Aranda, J;Chiesa, G;Mungianu, D;Bernardo-Seisdedos, G;Eftekharzadeh, B;Gairi, M;Pierattelli, R;Felli, IC;Diercks, T;Millet, O;Garcia, J;Orozco, M;Crehuet, R;L...
NATURE COMMUNICATIONS
2019-05-02 From 1,4-Disaccharide to 1,3-Glycosyl Carbasugar: Synthesis of a Bespoke Inhibitor of Family GH99 Endo-alpha-mannosidase
Lu, D(Lu, Dan);Zhu, S(Zhu, Sha);Sobala, LF(Sobala, Lukasz F.);Bernardo-Seisdedos, G(Bernardo-Seisdedos, Ganeko);Milet, O(Milet, Oscar);Zhang, YM(Zhang, Yongmin);Jimenez-Barbero, J(Jimenez-Ba...
ORGANIC LETTERS
2018-12-07 Bioengineered PBGD variant improves the therapeutic index of gene therapy vectors for acute intermittent porphyria
Serrano-Mendioroz, I(Serrano-Mendioroz, Irantzu);Sampedro, A(Sampedro, Ana);Serna, N(Serna, Naroa);de Salamanca, RE(Enriquez de Salamanca, Rafael);Sanz-Parra, A(Sanz-Parra, Arantza);Corrales...
HUMAN MOLECULAR GENETICS
2018-11-01 Directional coupling of oligodendrocyte connexin-47 and astrocyte connexin-43 gap junctions
Fasciani, I(Fasciani, Ilaria);Pluta, P(Pluta, Paula);Gonzalez-Nieto, D(Gonzalez-Nieto, Daniel);Martinez-Montero, P(Martinez-Montero, Paloma);Molano, J(Molano, Jesus);Paino, CL(Paino, Carlos ...
GLIA
2018-11-01 Repurposing ciclopirox as a pharmacological chaperone in a model of congenital erythropoietic porphyria
Urquiza, P(Urquiza, Pedro);Lain, A(Lain, Ana);Sanz-Parra, A(Sanz-Parra, Arantza);Moreno, J(Moreno, Jorge);Bernardo-Seisdedos, G(Bernardo-Seisdedos, Ganeko);Dubus, P(Dubus, Pierre);Gonzalez, ...
SCIENCE TRANSLATIONAL MEDICINE
2018-09-19 Structural basis of pyrrole polymerization in human porphobilinogen deaminase
Pluta, P(Pluta, Paula);Roversi, P(Roversi, Pietro);Bernardo-Seisdedos, G(Bernardo-Seisdedos, Ganeko);Rojas, AL(Rojas, Adriana L.);Cooper, JB(Cooper, Jonathan B.);Gu, S(Gu, Shuang);Pickersgil...
BIOCHIMICA ET BIOPHYSICA ACTA
2018-09-01 Structural basis and energy landscape for the Ca2+ gating and calmodulation of the Kv7.2 K+ channel
Bernardo-Seisdedos, G(Bernardo-Seisdedos, Ganeko);Nunez, E(Nunez, Eider);Gomis, C(Gomis, Carolina);Malo, C(Malo, Covadonga);Villarroel, A(Villarroel, Alvaro);Millet, O(Millet, Oscar)
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
2018-03-06 Disulfide driven folding for a conditionally disordered protein
Fraga, H(Fraga, Hugo);Pujols, J(Pujols, Jordi);Gil-Garcia, M(Gil-Garcia, Marcos);Roque, A(Roque, Alicia);Bernardo-Seisdedos, G(Bernardo-Seisdedos, Ganeko);Santambrogio, C(Santambrogio, Carlo...
SCIENTIFIC REPORTS
2017-12-05 In Vitro Approach To Identify Key Amino Acids in Low Susceptibility of Rabbit Prion Protein to Misfolding
Erana, H(Erana, Hasier);Fernandez-Borges, N(Fernandez-Borges, Natalia);Elezgarai, SR(Elezgarai, Saioa R.);Harrathi, C(Harrathi, Chafik);Charco, JM(Charco, Jorge M.);Chianini, F(Chianini, Fra...
JOURNAL OF VIROLOGY
2017-12-01 Missense UROS mutations causing congenital erythropoietic porphyria reduce UROS homeostasis that can be rescued by proteasome inhibition
Blouin, JM(Blouin, Jean-Marc);Bernardo-Seisdedos, G(Bernardo-Seisdedos, Ganeko);Sasso, E(Sasso, Emma);Esteve, J(Esteve, Julie);Ged, C(Ged, Cecile);Lalanne, M(Lalanne, Magalie);Sanz-Parra, A(...
HUMAN MOLECULAR GENETICS
2017-04-15 Contribution of Shape and Charge to the Inhibition of a Family GH99 endo-alpha-1,2-Mannanase
Petricevic, M(Petricevic, Marija);Sobala, LF(Sobala, Lukasz F.);Fernandes, PZ(Fernandes, Pearl Z.);Raich, L(Raich, Lluis);Thompson, AJ(Thompson, Andrew J.);Bernardo-Seisdedos, G(Bernardo-Sei...
JOURNAL OF THE AMERICAN CHEMICAL SOCIETY
2017-01-25 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:
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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.
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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.
