ALS and frontotemporal lobar degeneration (FTLD) cases with C9ORF72 expansion were observed not to contain protein aggregates comprised of the C9ORF72 protein [10], [11] though TDP-43 inclusions were observed and p62 was suggested to be the major disease protein since p62-immunoreactive neuronal cytoplasmic inclusions were found in the cerebral cortex, basal ganglia, hippocampus, and cerebellum [6], [9], [12]. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.