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- W2994891117 abstract "Myoclonus dystonia (DYT11) is a movement disorder caused by loss-of-function mutations in SGCE and characterized by involuntary jerking and dystonia that frequently improve after drinking alcohol. Existing transgenic mouse models of DYT11 exhibit only mild motor symptoms, possibly due to rodent-specific developmental compensation mechanisms, which have limited the study of neural mechanisms underlying DYT11. To circumvent potential compensation, we used short hairpin RNA (shRNA) to acutely knock down Sgce in the adult mouse and found that this approach produced dystonia and repetitive, myoclonic-like, jerking movements in mice that improved after administration of ethanol. Acute knockdown of Sgce in the cerebellum, but not the basal ganglia, produced motor symptoms, likely due to aberrant cerebellar activity. The acute knockdown model described here reproduces the salient features of DYT11 and provides a platform to study the mechanisms underlying symptoms of the disorder, and to explore potential therapeutic options." @default.
- W2994891117 created "2019-12-26" @default.
- W2994891117 creator A5025615169 @default.
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- W2994891117 creator A5091286843 @default.
- W2994891117 date "2019-12-23" @default.
- W2994891117 modified "2023-10-09" @default.
- W2994891117 title "Acute cerebellar knockdown of Sgce reproduces salient features of myoclonus-dystonia (DYT11) in mice" @default.
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- W2994891117 doi "https://doi.org/10.7554/elife.52101" @default.
- W2994891117 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/6959989" @default.
- W2994891117 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/31868164" @default.
- W2994891117 hasPublicationYear "2019" @default.
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