Most studied ALS/FTD-linked mutations in p62 are located in the promotor region and result in reduced p62 protein expression [182] or impair the recognition of ubiquitin or LC3 by p62 and thus impact cargo delivery to the autophagosome, always leading to a loss-of-function [168,177,183]. The gene discussed is SQSTM1; the disease is amyotrophic lateral sclerosis.