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Most relevant scientific articles
• romá-mateo, c., aGuaDo, c., Garcia-Gimenez, J.l., ibañez-cabellos, s., seco-cerVera, m. pallarDo, F.V., KnecHt, e., sanz, p . “Increased oxidative stress and impairment of antioxidant systems in Lafora disease models” . Mol . Neurobiol . PMID: 24838580 (2014) .
• ratHtHaGala m, breWer K.m., parKer m.W., sHerWooD a.r., WonG b.K., Hsu s., briDGes t.m., paascH b.c, Hellman l.m, HusoDo s., meeKins D.a., taylor a.o., turner b.D., auGer K.D., DuKHanDe V.V., cHaKraVartHy s., sanz p., WooDs V.V., li s ., Vander Kooi C .W . and Gentry M .S . “Structural Mechanism of Lafo- rin Function in Glycogen Dephosphorylation and Lafora Disease” . Molecular Cell . PMID: 25544560 (2014) .
Highlights
Institution: Agencia Estatal Consejo Superior de Investigaciones Científicas Contact: Instituto de Biomedicina de Valencia, CSIC · C/ Jaime Roig, 11 . 46010 Valencia . Phone: (+34) 96 339 17 60
• We have demonstrated that in fibroblasts from Lafora disease (LD) patients there is increase oxidative stress, due probably to a mitochondrial dysfunction . These results were confirmed in mouse models of Lafora disease, suggesting that oxidative stress should be considered as a new parameter in the pathophysiology of LD (Romá-Mateo et al ., 2014 Mol . Neurobiol, PMID: 24838580) .
• We have solved the crystal structure of laforin. This protein has two characteristic structural domains, a carbohydrate binding module (CBM) and a dual specificity phosphatase domain (DSP) . The solved structure allows the localization of the amino acid residues that are mutated in LD patients y their pathologic potential (Raththagala et al ., 2014, Mol Cell, PMID: 25544560) .
• We have presented a patent on new AMP-activated protein kinase (AMPK) activators. Due to the importance of AMPK in metabolic, cardiovascular, inflammatory and neurological diseases, we expect to attract pharmaceutical companies interested in developing these compounds (Castro et al ., 2014 P201431364) .
• In collaboration with the groups of Dr. Knecht and Pallardó, we have given an R+D service to the Khondrion Company, to evaluate the antioxidant capacity of two compounds from the company and the potential benefits on fibroblasts from LD patients .
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