Fig. 1.

The nuclear pore complex (NPC) is a massive structure comprised of 33 known protein components organized into five main structural domains–the cytoplasmic filaments (yellow), the inner and outer nuclear rings (green), the transmembrane components (purple), the nuclear core components (blue), and the nuclear basket (red) (A). Several nuclear pore components have been found to be disrupted in cases of ALS (colored in black)–Gle1, GP210, NDC1, Nup107, Nup133, Nup153, Nup50, Nup62, Nup98, POM121, RanBP2, and Tpr (B). Nup98 and Nup62 are disrupted in Alzheimer’s disease (C). Cases of Huntington’s disease show disruption of Nup62, Nup88, and Tpr (D). Miscellaneous juvenile neurodegenerative disorders also display disruption in nuclear pore components including ALADIN, Gle1, Nup214, Nup62, Nup88, and RanBP2, many involving a direct mutation in the gene encoding that NPC component (E)