Figure 2. Schematic representation of the functional domains of TDP-43.
TDP-43 is a 414-amino acid RBP with functions in transcriptional repression, splicing, and translational regulation. The vast majority of ALS-associated mutations occur within the C-terminal glycine rich PrLD. Table only includes data on studied links between ALS mutations and SG association in cellular models. Mutations are marked by red arrows and letters corresponding to table entries. NLS = Nuclear localization signal; RRM = RNA recognition motif; NES = Nuclear export signal; Tan shading = PrLD.