The comparison of data from proteomic analysis in myotilinopathy, filaminopathy and desminopathy revealed that desmin, filamin C, myotilin, N-RAP, Xin, Xirp2, αB-crystallin, and nestin were always over-represented in aggregate samples irrespective of the MFM subtype, although differences in ratios and proportions were detected. Here, DES is linked to Distal myotilinopathy.