TARDBP and amyotrophic lateral sclerosis: Evidence of neuronal apoptosis has been reported in ALS and FTLD-TDP cases, with elevated levels of activated caspase-3 seen in the spinal cord and brain (Martin, 1999; Su et al., 2000) and caspase-3 being identified as the protease responsible for TDP-43 fragmentation (Zhang et al., 2007).