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. Author manuscript; available in PMC: 2021 Feb 21.
Published in final edited form as: ACS Chem Biol. 2020 Jan 13;15(2):485–493. doi: 10.1021/acschembio.9b00958

Figure 1.

Figure 1.

The RNA that causes DM2, r(CCUG)exp, has multiple modes of toxicity. (A) r(CCUG)exp in intron 1 of CNBP folds into a hairpin displaying a periodic array of internal loops that sequester MBNL1. Sequestration of MBNL1 by r(CCUG)exp causes (B) pre-mRNA splicing defects, for example increasing IR exon 11 exclusion.