Figure 11.

Model of CFTR exon 9 splicing regulation by TDP-43 and the CELF proteins, ETR-3 and CUG-BP1. Large circles with dashed lines represent the spliceosomal complex, other circles represent splicing proteins. See the ‘Discussion’ section. (A) Exon 9 inclusion through U2AF⁶⁵ interaction. (B) ETR-3 (TDP-43) prevents U2AF⁶⁵ interaction, which blocks exon 9 inclusion. (C) Intermediate protein may help ETR-3 prevent U2AF⁶⁵ interaction and exon 9 inclusion. (D) CUG-BP1 binding does not hamper U2AF⁶⁵ interaction or exon 9 inclusion.