In 5–10% of patients with ALS, mutations occur in the genes coding for TAR DNA-binding protein 43 (TDP-43) and DNA/RNA-binding protein fused-in sarcoma (FUS) [69,70,71], and both corresponding proteins interact with the transcription-coupled nucleotide excision repair machinery [72]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.