Both DM1 and DM2 phenotypes correlate with MBNL downregulation and CELF1 upregulation, which leads to splicing misregulation of MBNL and CELF1 target pre-mRNAs in neuronal and cardiac muscle cells [182,183,184,185,186,187,188]. Here, CELF1 is linked to myotonic dystrophy type 1.