Mutations in the nuclear matrix protein matrin-3 can cause fALS30 and autosomal dominant distal myopathy with vocal cord and pharyngeal weakness;31, 32 matrin-3 interacts with TDP-43, FUS and other RBPs.33 Matrin-3 also showed cytoplasmic mis-localization in MCF 7 cells expressing mSigR1 (Figures 7c and d). This evidence concerns the gene TARDBP and autosomal dominant distal myopathy.