The presence of TDP-43-regulated cryptic exon splicing events in human post-mortem brain has been investigated in ALS, FTLD-TDP, and more recently, AD [6, 13, 23, 25, 27, 43, 49]; however, studies investigating whether neuronally enriched cryptic exons accumulate in the most common TDP-43 proteinopathy, LATE-NC, are lacking. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.