FHL 1 is beneficial in reducing muscle wasting in dystrophic FRG1 mice

Abstract

Four and a half LIM 1 (FHL1) is a protein highly expressed in skeletal muscle, and we have previously reported that FHL1 can promote myoblast fusion in vitro and skeletal muscle hypertrophy in vivo by activating the calcineurin/NFATc1 pathway. Therefore FHL1 has potential as a therapeutic target for muscle diseases that display a myoblast fusion defect and/or progressive muscle wasting. The focus of this study was to determine if FHL1 can reduce muscle wasting in the dystrophic FRG1 mouse. FRG1 is one of several candidate genes for Facioscapulohumeral muscular dystrophy and FRG1 transgenic mice show resemblance to this disease clinically and pathologically. The dystrophy in FRG1 mice is char..