Matrin-3 regulates transcription and stability of several RBPs including TDP-43 and FUS;33MATR3 mutations cause ALS and distal myopathy.30, 31, 32 Recently, mice over-expressing human matrin-3 were reported to develop muscular atrophy and altered spinal cord distribution of matrin-3 protein.54 Consistent with previous reports30, 31, 32 on human matrinopathy, we observed both cytoplasmic and nuclear matrin-3 accumulation in E102Q-SigR1 over-expressing cells, along with the aggregation of other RBPs relevant to ALS (TDP-43 and FUS). This evidence concerns the gene TARDBP and distal myopathy.