Research showed that the location of CELF1 plays an important role as overexpression of CELF1 in the nucleus leads to moderate histologic defects, such as myofiber variability, hyperpigmentation, myofiber necrosis, and severe myopathy, while overexpression of cytoplasmic CELF1 presented only minor myofiber variability [26,38]. Here, CELF1 is linked to myopathy.