Since Bmp7 was highly expressed in P0 mouse forelimb muscle and since addition of BMP-7 to forelimb organ cultures resulted in an increase in the numbers of myofibers with centrally localized nuclei, we hypothesized that lowering the expression level of Bmp7 should have beneficial effects on the Fbn2 null myopathy. Here, FBN2 is linked to myopathy.