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. 2018 Feb 1;10:376–386. doi: 10.1016/j.omtn.2017.12.012

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© 2018 The Authors

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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5′ Trans-Splicing of Lmna Improves the Nuclear Phenotype of Mutant Myotubes

(A and B) Immunofluorescence analysis of mutant myotubes that were non-transduced (NT) or transduced with AAV2/9-PTM-0, -PTM-BD3, and -PTM-BD3inv. Cells were differentiated and transduced for 7 days and then stained with lamin A/C (green)/emerin (red) (A) or FLAG (green)/Lamin B1 (red) (B). Nuclei were stained with DAPI (blue). Arrows point to repaired nuclei. Scale bars: 10 μm. (C) Representative confocal images of DAPI-stained myotubes that were either non-transduced (NT) or transduced with AAV2/9-PTM-BD3, and scatterplot showing the distribution and the mean size of nuclei long axis (NT: n = 107; PTM-BD3: n = 137). Error bars correspond to SEM.