Using a mouse model, researchers at IRB Barcelona, located in the Barcelona Scientífic Park, have demonstrated a link between abnormal sugar accumulation and the neuronal degeneration characteristic of Lafora disease. The study may contribute to understanding the origin and progression of Lafora and other neurodegenerative pathologies.

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Short-term energy storage in animal cells is usually achieved through the accumulation of glucose, in the form of long and branched chains, known as glycogen. But when this accumulation happens in neurons it is fatal, causing them to degenerate. This neuronal deterioration and death associated with glycogen accumulation is the hallmark of an extremely rare and progressive type of epilepsy known as Lafora disease (LD). The journal EMBO Molecular Medicine has just published online the new insights into LD provided by a team of Spanish researchers headed by Joan J. Guinovart, director of the IRB Barcelona and Professor at the University of Barcelona (UB), in collaboration with Agnès Gruart and José M. Delgado at the Division of Neurosciences at the Pablo Olavide University in Seville, and Eduardo Soriano, Professor at the UB and researcher at IRB Barcelona.

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