Nemaline myopathy (NM) is a genetic muscle disorder, notably caused by mutations in the NEB gene (NEB-NM). Here we investigated the efficacy of a four-week Mavacamten (myosin ATPase inhibitor) treatment using a NEB-NM mouse model. After the four weeks, soleus muscles were extracted, muscle fibres were isolated, and a global untargeted proteomics approach was employed. As presented in the data set, a lot of various proteins were affected by the treatment.