Frontotemporal lobar degeneration with TDP‐43 inclusions (FTLD‐TDP) is a fatal neurodegenerative disease distinguished by the accumulation of inclusions of hyperphosphorylated and ubiquitinated TAR DNA‐binding protein of 43 kD (TDP‐43), in glia and neurons. The gene discussed is TARDBP; the disease is neurodegenerative disease.