Figure 2.

Schematic of TDP-43 and FUS protein structure. TDP-43 and FUS, both have a Prion-like domain, nuclear localization signal, nuclear export signal and RNA recognition motif (RRM). FUS has an additional RRM as well as a Zinc finger domain. Major familial mutations are indicated. In contrast to FUS, disease-linked TDP-43 mutations are clustered in the Glycine-rich C-terminal domain; whereas FUS disease-linked mutations are mainly clustered in the nuclear localization signal domain.