In both myopathies and neurodegenerative diseases besides normal TDP-43, TDP-43 positive aggregates contain abnormal TDP-43 CTF, phosphorylated and highly ubiquitinated species (Weihl et al., 2008; Küsters et al., 2009; Olivé et al., 2009; Lain et al., 2011; Prasad et al., 2019). This evidence concerns the gene TARDBP and myopathy.