We chose to study TDP43 since abnormal TDP43 accumulations are a hallmark pathology of FTD/ALS and because mutations in TARDBP cause dominantly inherited familial forms of FTD/ALS; defective TDP43 metabolism is therefore believed to be central to most forms of FTD/ALS [30, 51]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.