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4-PBA and metformin decrease sensitivity to PTZ-induced seizures in a malin knockout model of Lafora disease

Lafora disease (LD) is a rare adolescent-onset progressive myoclonic epilepsy caused by loss-of-function mutations either in the EPM2A gene encoding laforin or in the EPM2B gene encoding malin. Mouse models with deletion in the Epm2a or Epm2b gene display intracellular aggregates of polyglucosans (L...

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Publicado en:Neuroreport
Autores principales: Sánchez-Elexpuru, Gentzane, Serratosa, José M., Sanz, Pascual, Sánchez, Marina P.
Formato: Artigo
Lenguaje:Inglês
Publicado: 2017
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Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC5491969/
https://ncbi.nlm.nih.gov/pubmed/28181916
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1097/WNR.0000000000000751
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