Data from: Valproic acid is protective in cellular and worm models of oculopharyngeal muscular dystrophy

Aida Abu-Baker, Alex Parker, Siriram Ramalingam, Janet Laganiere, Bernard Brais, Christian Neri, Patrick Dion & Guy Rouleau
Objective: To explore valproic acid (VPA) as a potentially beneficial drug in cellular and worm models of oculopharyngeal muscular dystrophy (OPMD). Methods: Using a combination of live cell imaging and biochemical measures, we evaluated the potential protective effect of VPA in a stable C2C12 muscle cell model of OPMD, in lymphoblastoid cell lines derived from patients with OPMD and in a transgenic Caenorhabditis elegans OPMD model expressing human mutant PABPN1. Results: We demonstrated that VPA...
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