Fig. 8: A working model of miR-322/-503 in DM1.

miR-322/-503, required by normal myoblast differentiation, was downregulated in DM1 myoblast differentiation and patient serums. Ectopic miR-322/-503 expression rescued myoblast differentiation defects, ribonuclear foci, and aberrant alternative splicing in DM1 myoblast mainly through targeting the expanded CUG repeats. Meanwhile, Celf1 as an alternative miR-322/-503 target only accounts for the partial rescue of myoblast differentiation defects. This figure was drawn with Pathway Builder 2.0.