Mutations inTARDBP that result in TDP-43 aggregation and neuropathology have been observed in distinct neurodegenerative diseases, known as TDP-43 proteinopathies.3,4 Various studies have identified a subset of amyotrophic lateral sclerosis (ALS) patients that possessTARDBP mutations, suggesting that TDP-43 gain of toxic function or loss of function is a causative factor in sporadic and/or familial ALS.4–6 Mechanistic studies would be greatly facilitated by the availability of high-quality antibodies. Here, TARDBP is linked to neurodegenerative disease.