In 2006, TDP-43 emerged as a prime component of ubiquitinated, insoluble inclusions found in the brains of patients diagnosed with amyotrophic lateral sclerosis (ALS), a progressive motor syndrome that causes muscle weakness and atrophy, and frontotemporal dementia (FTD), a disease involving a variety of cases with behavioral and language impairment that is often accompanied by atrophy of the frontal and temporal lobes [7, 8]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.