In C9orf72-related ALS TDP-43 proteinopathy is present, but additional inclusions are p62/sequestosome-1 and ubiquitin positive, yet devoid of TDP-43 (Al-Sarraj et al., 2011; Cooper-Knock et al., 2012; Mackenzie et al., 2014). The gene discussed is SQSTM1; the disease is amyotrophic lateral sclerosis.