Dementia and the TAR DNA binding protein 43

Neurodegenerative dementias are characterized by the deposition of soluble aberrant protein(s). β-Amyloid, the microtubule-associated protein tau, and α-synuclein are the most well-recognized proteins associated with neurodegenerative dementias. Recently, another protein, the transactive response DNA binding protein of 43 kDa (TDP-43), was discovered to play a major role in frontotemporal lobar degeneration (FTLD), a group of diseases associated with young-onset dementia. TDP-43 has now been linked to more than 10 diseases, including amyotrophic lateral sclerosis (ALS).