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. Here, TARDBP is linked to frontotemporal dementia.