FUS and amyotrophic lateral sclerosis: Interestingly, the majority of ALS-causing mutations in FUS are found not in the PrLD but in the C-terminal region (Da Cruz and Cleveland, 2011; Kwiatkowski et al., 2009; Vance et al., 2009), where they disrupt a proline-tyrosine (PY) nuclear localization signal (Lee et al., 2006; Suel et al., 2008; Zhang and Chook, 2012).