However, when the ratio of CNBP/MBNL1 is examined, this correlation is lost, perhaps because of the involvement of additional RBPs such as RBFOX1/2 in DM2 pathogenesis (16), although RBFOX1 and RBFOX2 have similar expression profiles to MBNL1 across muscles, perhaps indicating yet unknown mechanisms of susceptibility. Here, MBNL1 is linked to myotonic dystrophy type 2.