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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. 4. — (CAG)7-ASO reverses CUGexp-RNA toxicity in immortalized muscle DM1 cells. (A) Percentage of immortalized DM1 myoblasts containing nuclear CUGexp-RNA aggregates (foci) following 2′OMe-(CAG)7 treatment (n=4). Data are expressed as mean±s.e.m. Comparison with Mann-Whitney test; ***P<0.001. (B) FISH immunofluorescence staining using a Cy3-(CAG)7 probe and an anti-MBNL1 antibody to detect nuclear foci (red) and MBNL1 (green), respectively, in immortalized DM1-cl5 myoblasts treated with 2′OMe-(CAG)7 ASOs compared with non-treated (NT) cells. (C) FISH experiment to detect CUGexp-RNA foci (red) in the nucleus (blue) of myotubes derived from converted DM1 fibroblast cell lines treated with 2′OMe-(CAG)7 ASOs. (D) Quantification of MBNL1 exon 7, LDB3 exon 11 and BIN1 exon 11 splicing profiles determined by RT-PCR in myotubes cultures of converted Ctrl and DM1 fibroblast cell lines treated or not with 2′OMe-(CAG)7 ASOs (n=8, except for BIN1 where n=6). Data are expressed as mean±s.e.m. Comparison with one-way ANOVA with Newman-Keuls post-test; **P<0.01, ***P<0.001; ns, not significant.