TDP-43 is implicated in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD-U) where TDP-43 is found in large insoluble granules in the cytoplasm, but the pathogenesis of these granules is not clear. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.