In DM1, CUGBP1 is hyper-phosphorylated by protein kinase C (PKC) and its level increased, whereas MBNL1 and MBNL2 are bound abundantly to mutated transcripts and retained in the nucleus, co-localised with RNA foci and therefore are not accessible for a correct splicing process in other pre-mRNAs [41,42]. Here, CELF1 is linked to myotonic dystrophy type 1.