Notably, TDP-43 is also localized in the intracytoplasmic ubiquitinated inclusions of sporadic amyotrophic lateral sclerosis (ALS), a motor neuron disease often associated with FTLD-U [3,6], and mutations in TDP-43 have been linked to ALS [7-10]. The gene discussed is TARDBP; the disease is sporadic amyotrophic lateral sclerosis.