Consistent with compromised autophagy, C9ALS/FTD patients characteristically exhibit ubiquitin and p62/sequestosome-1 positive, TDP-43 negative, inclusions in the cerebellum and hippocampus (Al-Sarraj et al., 2011; Cooper-Knock et al., 2012; Mahoney et al., 2012). This evidence concerns the gene TARDBP and frontotemporal dementia.