In 2006, the predominately nuclear TAR DNA-binding protein 43 (TDP-43) was identified as a key component of the insoluble and ubiquitinated inclusions in the brains of patients suffering from amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD or FTLD-TDP) diseases [190, 191]. The gene discussed is TARDBP; the disease is frontotemporal dementia.