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Recent transgenic studies suggest that FRG1 over-expression results in the mis-splicing of skeletal troponin and myotuburlarin related protein-1 and a muscular dystrophy phenotype (Gabellini et al. 2006). We are investigating FRG1 expression during myoblast growth and differentiation in vitro. We are also studying potential interactions between FRG1 and the D4Z4 repressor complex; and we are developing an in vitro splicing assay to analyze FRG1’s role in MTMR1 splicing.