C9ORF72-related ALS is neuropathologically characterized by the presence of TDP-43-negative, ubiquitin- and p62-positive and intranuclear and cytoplasmic neuronal inclusions that contain DPR proteins.30 DPRs are peptides derived from unconventional repeat-associated non-ATG translation of repeated RNA foci transcribed from the HRE and represent C9ORF72-ALS biomarkers.31,32 Different types of DPRs can be isolated via chromatography and quantified via Meso Scale Discovery-based immunoassays. Here, TARDBP is linked to amyotrophic lateral sclerosis.