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lüll Impaired autophagy in Lafora disease Knecht E; Aguado C; Sarkar S; Korolchuk VI; Criado-Garcia O; Vernia S; Boya P; Sanz P; Rodriguez de Cordoba S; Rubinsztein DCAutophagy 2010[Oct]; 6 (7): 991-3Lafora disease (LD) is a progressive, lethal, autosomal recessive, neurodegenerative disorder that manifests with myoclonus epilepsy. LD is characterized by the presence of intracellular inclusion bodies called Lafora bodies (LB), in brain, spinal cord and other tissues. More than 50 percent of LD is caused by mutations in EPM2A that encodes laforin. Here we review our recent findings that revealed that laforin regulates autophagy. We consider how autophagy compromise may predispose to LB formation and neurodegeneration in LD, and discuss future investigations suggested by our data.|Animals[MESH]|Autophagy/*physiology[MESH]|Carrier Proteins/genetics/metabolism[MESH]|Humans[MESH]|Lafora Disease/*physiopathology[MESH]|Mice[MESH]|Proteasome Endopeptidase Complex/metabolism[MESH]|Protein Tyrosine Phosphatases, Non-Receptor/genetics/metabolism[MESH]|Ubiquitin-Protein Ligases[MESH] |