Metal dyshomeostasis is implicated in a number of neurodegenerative diseases (Sofic et al., 1988; Connor et al., 1992; Lovell et al., 1998), but whether metals play a role in the degeneration of neurons in TDP-43 associated forms of diseases such as FTLD-U and ALS is unknown. Here, TARDBP is linked to amyotrophic lateral sclerosis.