TARDBP and amyotrophic lateral sclerosis: This notion had been especially based in a prior study on two findings: (i) a frequency of 0.12% (3/2442) and 0.14% (5/3674) HTT-carriers, respectively, in FTD/ALS cohorts compared to 0.03% (10/31372) in the general population; and (ii) both FTD/ALS-typical TDP-43-pathology and polyQ-inclusions without evidence of striatal degeneration in autopsy of two HTT-carriers with an ALS-phenotype [1].