Progranulin mediates caspase-dependent fragmentation of TDP-43 generating a heterogenous population of C-terminal fragments (CTFs) of ~ 25 kDa and ~35 kDa that have been found in the cytoplasmic inclusions of some FTLD and ALS patients as well as in model animals(Neuman et al., 2006; Zhang et al., 2007; Igaz et al., 2008; Li et al., 2015). Here, TARDBP is linked to amyotrophic lateral sclerosis.