Although the detail pathological mechanism of the SYG/SYGQ-NES protein remains unknown and requires further study, recent evidence suggests that ALS-linked RNA-binding proteins, such as TDP-43 and FUS, are mislocated to the cytoplasm and might affect target RNAs and other RNA-binding proteins, resulting in impairment of RNA metabolism and RNA quality control mechanisms, such as those associated with SGs, during neurodegeneration1,41. This evidence concerns the gene FUS and amyotrophic lateral sclerosis.