TDP-43 pathology is present in most sporadic cases and in genetic ALS associated with mutations in chromosome 9 open reading frame 72 (C9orf72), TARDBP, optineurin (OPTN), sequestosome 1 (SQSTM1), ubiquilin- 2 (UBQLN2), and TANK binding kinase 1 (TBK1) [43]. This evidence concerns the gene OPTN and amyotrophic lateral sclerosis.