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Title

Regulation of glycogen synthesis by the laforin-malin complex is modulated by the AMP-activated protein kinase pathway

AuthorsSolaz-Fuster, Maria Carmen ; Gimeno-Alcañiz, José Vicente; Ros, Susana; Fernández-Sánchez, Maria Elena; García-Fojeda, Belén; Criado-García, Olga ; Vilchez, David; Domínguez, Jorge; Ros, Susana; García-Rocha, Mar; Sánchez-Piris, Maribel; Aguado, Carmen; Knecht, Erwin; Serratosa, José María; Sanz, Pascual ; Rodríguez de Córdoba, Santiago
Issue Date1-Mar-2008
PublisherOxford University Press
CitationHuman Molecular Genetics 17(5):667-78(2008)
AbstractLafora progressive myoclonus epilepsy (LD) is a fatal autosomal recessive neurodegenerative disorder characterized by the presence of glycogen-like intracellular inclusions called Lafora bodies. LD is caused by mutations in two genes, EPM2A and EPM2B, encoding respectively laforin, a dual-specificity protein phosphatase, and malin, an E3 ubiquitin ligase. Previously, we and others have suggested that the interactions between laforin and PTG (a regulatory subunit of type 1 protein phosphatase) and between laforin and malin are critical in the pathogenesis of LD. Here, we show that the laforin–malin complex downregulates PTG-induced glycogen synthesis in FTO2B hepatoma cells through a mechanism involving ubiquitination and degradation of PTG. Furthermore, we demonstrate that the interaction between laforin and malin is a regulated process that is modulated by the AMP-activated protein kinase (AMPK). These findings provide further insights into the critical role of the laforin–malin complex in the control of glycogen metabolism and unravel a novel link between the energy sensor AMPK and glycogen metabolism. These data advance our understanding of the functional role of laforin and malin, which hopefully will facilitate the development of appropriate LD therapies
Description12 páginas, 8 figuras -- PAGS nros. 667-678
Publisher version (URL)http:dx.doi.org/10.1093/hmg/ddm339
URIhttp://hdl.handle.net/10261/57424
DOI10.1093/hmg/ddm339
ISSN0964-6906
E-ISSN1460-2083
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