Becker Muscular Dystrophy (BMD) is a rare genetic disease that results in the expression of a truncated, partially functional isoform of dystrophin leading to progressive weakness and loss of muscle function. Our lab previously developed and characterized a mouse model of BMD, termed bmx, which carries a deletion in dystrophin exons 45-47. Skeletal muscle from bmx mice exhibits markedly reduced dystrophin protein and elevated levels of dystrophin-targeting microRNAs (DTMs). This increase in DTMs is likely a consequence of increased inflammatory signaling in bmx muscle, partially due to truncated dystrophin protein, which cannot fully protect against contraction-induced damage. We have also shown that treatment of dystrophin-null (mdx) mice with a selective, orally bioavailable inhibitor of fast skeletal myosin modestly reduces fast fiber contraction, providing protection against stress-induced injury. Here we test the hypothesis that inhibition of fast myosin with the inhibitor EDG-4131 can improve bmx muscle pathophysiology. Bmx mice treated with EDG-4131 from 6 to 12 weeks of age show reduced pathology and immune cell infiltration in treated muscles. EDG-4131-treated bmx mice also showed reduced levels of several inflammatory transcripts and microRNAs. Additionally, we tested a secondary hypothesis that treatment with EDG-4131 would increase dystrophin levels through downstream effects on DTMs. While we did not observe an increase in dystrophin levels, analysis of electropherogram (JESS) signal revealed an increase in peak height with corresponding reduction in peak width, indicative of a potential stabilization effect on key sarcomeric proteins (dystrophin, -actinin). Together, our data suggest EDG-4131 exerts a protective effect and reduces inflammatory signaling in bmx muscle. Ongoing investigations are exploring the mechanism by which this treatment may also stabilize structural proteins, potentially impacting sarcomeric proteins and proteosome activity.