There are several ALS-associated mutations: SOD-1 (superoxide dismutase), TAR DNA-binding protein 43 (TDP-43, transactive response DNA binding protein 43 kDa), C9orf72 (chromosome 9 open reading frame 72) protein, RNA-binding protein FUS/TLS (Fused in Sarcoma/Translocated in Sarcoma), etc. The multiple pathogenic mechanisms of ALS (including TDP-43 mutations) share cytoplasmic TDP-43 deposits as a common trait, which are thought to be critical in the degenerative process of motor neurons, and considered the final hallmark of ALS. This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.