In the 2007 edition of the Annals of Neurology, Lee and Trojanowski published a paper suggesting that a ubiquitinated version of a protein called TDP-43 was a pathological hallmark of sporadic ALS, ALS with dementia, and SOD1-negative familial ALS (Mackenzie et al., 2007). Here, TARDBP is linked to amyotrophic lateral sclerosis.