Fig. 1

Protein structure of transactive response DNA binding protein of 43 kDa (TDP-43). TDP-43 is a 414 amino acid protein with a nuclear localization sequence (NLS) followed by two RNA binding domains (RBD1 and RBD2), a nuclear export sequence (NES), and a glycine rich prion-like (GRD) C-terminus. The mutations reported to increase the risk of ALS (red), FTLD (blue), and AD (orange) are indicated