Over the past decade, many ALS-associated proteins, including UBQLN2, TDP-43, FUS, and C9orf72, have been identified as SG components, and it is hypothesized that SG formation may trigger neurodegenerative processes by sequestering critical RNA-binding proteins (RBPs) and factors [6,30,32,33]. This evidence concerns the gene UBQLN2 and amyotrophic lateral sclerosis.