ATXN3 and amyotrophic lateral sclerosis: The study by Cui et al. [170] subsequently showed that knockdown of ATXN3 resulted in a substantial increase in the accumulation and phosphorylation of TDP-43 C-terminal fragments, and that overexpression of ATXN3 reduced this phenotype, thereby demonstrating that ATXN3 modulation impacts important molecular hallmarks of ALS pathology in vitro.