Gys1 Antisense Therapy Prevents Disease-Driving Aggregates and Epileptiform Discharges in a Lafora Disease Mouse Model.

doi:10.1007/s13311-023-01434-9

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Article

Gys1 Antisense Therapy Prevents Disease-Driving Aggregates and Epileptiform Discharges in a Lafora Disease Mouse Model.

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics - 1 Oct 2023

Donohue Katherine J, Fitzsimmons Bethany, Bruntz Ronald C, Markussen Kia H, Young Lyndsay E A, Clarke Harrison A, Coburn Peyton T, Griffith Laiken E, Sanders William, Klier Jack, Burke Sara N, Maurer Andrew P, Minassian Berge A, Sun Ramon C, Kordasiewisz Holly B, Gentry Matthew S

Abstract excerpt

Patients with Lafora disease have a mutation in EPM2A or EPM2B, resulting in dysregulation of glycogen metabolism throughout the body and aberrant glycogen molecules that aggregate into Lafora bodies. Lafora bodies are particularly damaging in the brain, where the aggregation drives seizures with increasing severity and frequency, coupled with neurodegeneration. Previous work employed mouse genetic models to...

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PMID: 37700152
DOI: 10.1007/s13311-023-01434-9
PMCID: PMC10684475

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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