Figure 1. Schematic overview of ALS/FTD disease proteins known to play a role in the autophagy and endosomal–lysosomal pathways.
Sellier et al (2016) identified a novel complex consisting of C9ORF72, WDR41, and SMCR8 that serves as a GEF for RAB39b. This complex may play a modulatory role in the initiation of autophagy in certain cell types, but detailed molecular mechanisms remain unclear. Key functions of many other ALS/FTD disease proteins (in red) in some steps of the autophagy and endosomal–lysosomal pathways are also highlighted. MVB, multivesicular body.