TARDBP and amyotrophic lateral sclerosis: These findings are of likely translational significance since familial and sporadic ALS cases do express functional wildtype copies of TDP-43 with an intact NLS that are available for karyopherin-mediated nuclear import (Arai et al., 2006; Neumann et al., 2006; Sreedharan et al., 2008; Kabashi et al., 2008; Yokoseki et al., 2008).