The neuropathological features of TDP‐43 proteinopathy in ALS and FTD include nuclear to cytoplasmic mislocalization, posttranslational modifications such as ubiquitylation and phosphorylation, aggregation, and N‐terminal truncated C‐terminal TDP‐43 fragments (CTFs) (1, 4). The gene discussed is TARDBP; the disease is frontotemporal dementia.