The current study demonstrates that PQE Atx2 aggregation is deleterious to mRNA metabolism by sequestrating cytoplasmic DDX6 and impairing P-body assembly, whereas restoration of the P-body functionalities by addition of DDX6 may be a potential therapeutic approach for SCA2 as well as ALS. This evidence concerns the gene ATXN2 and amyotrophic lateral sclerosis.