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. 2017 Apr 1;10(4):487–497. doi: 10.1242/dmm.027367

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© 2017. Published by The Company of Biologists Ltd

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attributed.

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Fig. 5. — hU7-(CAG)15 reverses CUGexp-RNA toxicity in DM1 myoblast cell lines. (A) Differentiation of DM1-cl5 myoblast cell line expressing hU7-(CAG)15 antisense transcripts was assessed by desmin (green) and Hoechst (blue) immunostaining and compared with differentiated DM1-cl5 and Ctrl cells. (B) Fusion index of differentiated cultures of immortalized DM1 myoblasts transduced or not with hU7-(CAG)15 lentiviral vectors compared with Ctrl-cl4 (n=4 for each condition, >1000 nuclei counted per experiment). Data are expressed as mean±s.e.m. Comparison with Mann-Whitney test; *P<0.05. (C) Nuclear CUGexp-RNA aggregates in differentiated DM1-cl5 myoblast cell line expressing hU7-(CAG)15. (D) RT-PCR analysis of BIN1 exon 11, DMD exon 78 and LDB3 exon 11 splicing profiles in differentiated cultures of immortalized Ctrl-Cl4 and DM1-cl5 myoblasts transduced or not with hU7-(CAG)15 lentiviral vectors (n=6 for each condition). Data are expressed as mean±s.e.m. Comparison with one-way ANOVA with Newman-Keuls post-test; ***P<0.001.