Interestingly, the skein-like inclusions in spinal cord neurons of ALS patients which are positive for ubiquilin 2 also contain ubiquitin, optineurin, TDP-43 and FUS (Deng et al., 2011b; Williams et al., 2012), suggesting that the deregulation of protein clearance is linked to neurodegeneration in ALS patients. Here, UBQLN2 is linked to amyotrophic lateral sclerosis.