TAR DNA-binding protein 43 (TDP-43) is a nuclear proteinaccumulatingin intraneuronal cytoplasmic inclusions associated with amyotrophiclateral sclerosis, frontotemporal lobar degeneration with tau-negative/ubiquitin-positiveinclusions, and limbic-predominant age-related TDP-43 encephalopathy.Oligomerization of full-length TDP-43, driven by its N-terminal domain(NTD), is essential for its function, but aberrant self-assembly alsopromotes liquid–liquid phase separation and formation of solidinclusions. Here, TARDBP is linked to frontotemporal dementia.