In addition to sporadic forms, several mutations in different genes, including the C9orf72 (most frequent), granulin (GRN), valosin-containing protein (VCP), TARDBP, SQSTM1 (sequestome), DCTN1 (dynactin), and OPTN (optineurin) have been reported to be associated with TDP-43 proteinopathy [181,182,183,184,185,186,187,188,189,190]. This evidence concerns the gene TARDBP and proteostasis deficiencies.