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Interbio aims at promoting interdisciplinary and interregional research in the field of health & life sciences. Interbio can provide mobility support (transport & accommodation) for scientists interested in setting up collaborative research projects.
For more information, please contact your local WP3 coordinator.

Evaluation of mitochondrial beta-oxidation flavoenzymes in a cellular model of Friedreich ataxia

Partner A Member : Valencia
Name of principal investigator : PALAU
FirstName : Francesc
E-mail : fpalau@ibv.csic.es
Position : Staff
Affiliation Name : Instituto de Biomedicina de Valencia, CSIC
Road : C/ Jaume Roig, 11
Postbox : 46010
Town : Valencia
Country : Spain
Partner B Member : Lisboa
Name of principal investigator : GOMES
FirstName : Cláudio
E-mail : gomes@itqb.unl.pt
Position : Staff
Affiliation Name : Instituto de Tecnologia Quimica e Biologica, Universidade Nova de Lisboa
Road : Av. da Republica. E. A. N.
Postbox : 2784-505
Town : Oeiras
Country : Portugal
Request for financial support 1 scientist for 14 days (Partner A to Partner B)
Perspectives of extension of this common project to a more ambitious proposal to be submitted to funding agencies : Long term
Added value of the cooperation : Exchange of expertise

Summary of the proposal : Frataxin deficiency is the cause of Friedreich ataxia, a neurodegenerative disorder. Physiological function of frataxin in mitochondria has not been well understood and several hypotheses on the pathogenic mechanisms have been postulated. The Palau laboratory has described new interactions, by co-immunoprecipation assays, of Saccharomyces cerevisiaefrataxin orthologue Yfh1p with succinate dehydrogenase complex subunits Sdh1p and Sdh2pof the yeast mitochondrial electron transport chainand also with ETF-alpha and ETF-beta subunits from the electron transfer flavoprotein complex.Flavin adenine dinucleotide (FAD) is a prosthetic group that is covalently anchored to the SDHA subunit of Complex II and also acts as a cofactor to the ETF complex. We showed that riboflavin-derived cofactors, especially FAD, rescue cell growth and enzymatic activities of the electron transport chain, except complex II, in yfh1Δyeast. We suggested that improving in yeast growth is not related with complex II but might be related with the electron transfer flavoprotein (ETF) complex. The Gomes lab has been investigating ETF as well as other enzymes in the context of mitochondrial diseases affecting fatty acid metabolism, also in respect to the effects of riboflavin supplementation, and we have optimized procedures for protein and activity analysis in cell extracts.

At this point we are interested to understand how frataxin deficiency affects the activity of mitochondrial fatty acids beta oxidation enzymes. With this purpose we have developed a chronic model of this disease, with RNAi technology, generating stable lines deficient for frataxin in a neuronal human line of neuroblastoma (SH-SY5Y). Up to now we known that frataxin silencing leads to a slower cell growth, and the unique activity of electron transport chain perturbed was at the level of complex IV. This proporal aims at 1) Analyse the expression levels of fatty acid beta oxidation protein levels using immunoblotting and monoclonal antibodies and 2) determine the enzymatic activity of several fatty acid beta oxidation enzymes (e.g MCAD, VLCAD and ETF) in cellular extracts from frataxin deficient human neuroblastoma cell lines (SH-SY5Y).

Antonio PINEDA-LUCENA, Valencia
Claudio GOMES, Lisboa
Using metabonomics to understand fatty acid oxidation disease View proposal
Miriam ROYO EXPOSITO, Barcelona
Ivan HUC, Bordeaux
Exploring delta-aromatic carboxamide foldamers and alpha-peptides compatibility View proposal
Salvador VENTURA ZAMORA, Barcelona
GT4.- Proteomic analysis of cellular responses to protein-aggregation-mediated stress View proposal
Olivier GADAL, Toulouse
Marc MARTI-RENOM, Valencia
Integrating probabilistic maps and 3C-based data for determining high-resolution 3D structure of yeast chromosomes View proposal
Yann ASTIER, Lisboa
Josep SAMITIER, Barcelona
Nanochannels for single molecule studies View proposal
Jacqueline GRIMA PETTENATI, Toulouse
Jorge PINTO PAIVA, Lisboa
The MicroRNAs in Eucalyptus tension wood world View proposal
Royo BEATRIZ , Lisboa
Gómez MONTSERRAT, Toulouse
Molybdenum complexes containing chiral oxazoline ligands: coordination and catalysis View proposal
Fernando ROCH, Toulouse
Elio SUCENA, Lisboa
Studying functional diversification and evolution of regulatory sequences after a gene duplication event View proposal
Gilles GUICHARD, Bordeaux
Fernando ALBERICIO, Barcelona
High-Throughput Screening of one-bead/one-compound (OBOC) foldamer libraries against protein targets View proposal
Bruno BRUNO VICTOR, Lisboa
Xavier XAVIER DAURA, Barcelona
Molecular Simulations of Hemagglutinin from Influenza virus View proposal
Bernard MONSARRAT, Toulouse
WG1 Comparative proteome analysis of C. albicans strains SC5314 and CAI4 View proposal
Peter FALLER, Toulouse
Claudio GOMES, Lisboa
Structural and functional aspects of the Cu/Fe-amyloid-beta complex View proposal
Egea GUSTAVO, Barcelona
Elisabeth GENOT, Bordeaux
TGFbeta signaling in cardiovascular alterations associated with the Marfan syndrome View proposal
Cecilia ARRAIANO, Lisboa
Stabilome E coli View proposal
Muñoz-almagro CARMEN , Barcelona
Sa-leao RAQUEL, Lisboa
Invasiveness of pneumococcal serotypes and clones circulating in Catalonia during heptavalente conjugate vaccine period View proposal
Olga IRANZO, Lisboa
Rafel PROHENS, Barcelona
Study of the influence of peptide flexibility on metal ion coordination properties View proposal
Rita ABRANCHES, Lisboa
José maría SEGUÍ, Valencia
Investigating the subcellular trafficking of human recombinant proteins expressed in plants View proposal
Francesc PALAU, Valencia
Cláudio GOMES, Lisboa
Evaluation of mitochondrial beta-oxidation flavoenzymes in a cellular model of Friedreich ataxia View proposal
Jean-jacques TOULMÉ, Bordeaux
Claudio GOMES, Lisboa
Studies on human S100 protein-aptamer complexes View proposal
Justine BERTRAND-MICHEL, Toulouse
Gemma FABRIÀS, Barcelona
WP4 Sphingolipidomic technical exchange View proposal
Célia ROMÃO, Lisboa
Jordi juanhuix GIBERT, Barcelona
Introduction to the MX XALOC BL13 Beamline at ALBA View proposal

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