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. 2017 Jun 6;7:2843. doi: 10.1038/s41598-017-02829-3

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© The Author(s) 2017

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/.

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CUG and CCUG expansions cause Muscleblind-dependent missplicing. (A,B) Representative semi-quantitative RT-PCR showing inclusion of Fhos exon 16′ in flies expressing the indicated constructs in muscle (A) or heart (B) under the control of Mhc-Gal4 or GMH5-Gal4, respectively. Endogenous Rp49 was used for normalization. Percentage of exon 16′ inclusion, revealed that expression of long CUG or CCUG repeats in the fly muscle (C) or heart (D), favored increased use of this exon. qRT-PCR results of Serca exon 13 expression relative to Rp49 expression, confirmed that the use of this exon in the flies expressing the expanded repeats and the control flies is significantly different in muscle (E) and heart tissues (F). The histograms show the mean ± SEM. *p < 0.05, **p < 0.01, ***p < 0.001 (Student’s t-test).