We next asked whether nsP3-WT could alleviate neurodegenerative phenotypes in four Drosophila ALS models: two TDP43 models (overexpression of EGFP-TDP43 or the ALS-associated TDP43 (M337V) mutant, which enhances TDP43 cytoplasmic mislocalization, disrupts SG dynamics and causes more rapid neurodegeneration in Drosophila (20,67–69)), and two C9orf72 RAN translation models ((GGGGCC)28-GFP, and (GR)100), both of which express glycine–arginine DPRs that form large GR aggregates that cause extensive neurodegeneration and impair SG dynamics (20,24,70,71)). Here, SH2D3C is linked to amyotrophic lateral sclerosis.