TAR DNA-binding protein 43 (TDP-43), which pathologically accumulates in 97% of ALS patients (including in oligodendrocytes [7]), has been shown to be integral to normal oligodendrocyte function and myelin production [8], thereby raising the question as to how pathogenic TDP-43 in ALS may impact on normal oligodendrocyte function. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.