Also in agreement with defective autophagy caused by C9orf72 loss of function, C9ALS/FTD patients show specific ubiquitin and p62‐positive inclusions in the brain (Al‐Sarraj et al, 2011; Cooper‐Knock et al, 2012; Mackenzie et al, 2014) and our data show that loss of C9orf72 in cells and primary neurons mimics C9ALS/FTD p62 pathology (Fig 8). Here, SQSTM1 is linked to frontotemporal dementia.