TDP-43 UBQLN2-positive inclusions with or without UBQLN2 and TDP-43 mutations have also been consistently identified in ALS and FTD patients, with high levels of the UBQLN2 wild type or mutant enhancing the cytosolic accumulation of TDP-43 [148,149,150]. Here, UBQLN2 is linked to amyotrophic lateral sclerosis.