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. 2017 Nov 13;15(1):1–8. doi: 10.1080/15476286.2017.1384119

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© 2018 The Author(s). Published with license by Taylor & Francis Group, LLC

This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License (http://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited, and is not altered, transformed, or built upon in any way.

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Figure 4. — Overlapping and compensatory roles of MBNL1 and MBNL2. Total mRNA (upper panel) and e5-containing mRNA (lower panel) expression level of MBNL1 (left) and MBNL2 (right) transcripts in DM1 fibroblasts (∼1000 CUGs) with siRNA-mediated knock-down of MBNL1 or MBNL2 or both. AllStars negative control siRNA (Qiagen) was used as a control (siCtrl). Note that siRNA-mediated MBNL1 knock-down results in MBNL2 mRNA increase, but not vice-versa (upper panel). Also, combined knock-down of MBNL1 and MBNL2 augments e5 inclusion when compared to knock-down of either of the paralogs alone (lower panel). Also note that 1000 CUGs DM1 fibroblasts have already partially depleted basal level of functional MBNLs due to their sequestration by expanded CUG repeats. In non-DM1 fibroblasts the following values for e5 splicing were obtained, MBNL1: Mock 3.50% (±1.49), siCtrl 5.03% (± 0.74), siMBNL1 12.32% (±3.81); MBNL2: Mock 2.52% (±0.59), siCtrl 2.92% (±0.13), siMBNL1 18.45% (±1.06) (data not included in bar graphs). The data shown have not been previously published. Detailed preparation of the samples was described in³.