TARDBP and proteostasis deficiencies: This study utilizes the recently developed TDP-43A315T mouse model of FTLD/ALS (Wegorzewska et al., 2009) to assess whether the presence of a pathogenic form of TDP-43 affects metal ion homeostasis in vivo, and if present, to assess whether altered metal homeostasis may be associated with the symptoms of neuronal decline in an animal model of TDP-43 proteinopathy.