Only ten of the in total 76 proteins that were enriched in aggregates were previously described components of deposits in myotilinopathy, namely myotilin [4, 11], desmin [4, 11], filamin C [13, 20], αB-crystallin [4, 11], Xin [16], Xirp2 [16], plectin [11], dystrophin [4, 11], NBR1 [21] and p62 [15]. This evidence concerns the gene NBR1 and Distal myotilinopathy.