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. 2021 May 10;12(5):466. doi: 10.1038/s41419-021-03710-y

Scheme 1. Proposed cohesive pathomechanism of aberrant RBPs hoemostasis and autophagy in ALS8.

Scheme 1

P56S-VAPB aggregates alter early autophagy proteins ULK1, ATG5-ATG12; late autophagy protein STX17 and leads to the defects in both early and late steps of autophagy. P56S-VAPB mediated proteotoxic stress together with altered autophagy might contribute to dysregulations of several fALS-associated RBPs (TDP-43, FUS, Matrin 3) including the SGs and can initiate a vicious cycle. (Schematic image was generated with the BioRender software).