Subsequently, the ubiquitinated compact and skein-like inclusions, characteristic for amyotrophic lateral sclerosis (ALS) were also found to be composed of TDP-43 [5, 7], thereby providing strong evidence that both conditions are mechanistically linked and part of a clinico-pathological spectrum of disease, which can be subsumed as TDP-43 proteinopathies [8, 9]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.