In support of this, mutations in the autophagy receptors OPTN and SQSTM1 are causal of FTD and ALS and TBK1 promotes autophagy via phosphorylation of OPTN and SQSTM1 therefore enhancing the ubiquitin-binding abilities of both proteins [104,105]. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.