In addition to the typical TDP-43 pathology, C9ORF72-ALS/FTD shows specific DPR pathology from sense and antisense repeat expansion-containing transcripts, comprised of star shaped TDP-43 negative, tau-negative, ubiquitin-positive and p62-positive inclusions throughout the cerebellar cortex, neocortex, and hippocampus. This evidence concerns the gene SQSTM1 and amyotrophic lateral sclerosis.