TDP-43, a protein with dual functions as DNA-binding protein with nuclear export sequence and as RNA-binding protein, is found to be predominantly localized to the cytosol instead of the nucleus in ALS spinal MNs, found in punctate immunoreactive granules and as dense skeins (Kabashi et al., 2008). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.