LC3 fails to recognize p62 when ALS-associated L341V mutation occurs in such cells, whereas ubiquitinated proteins still bind to it, thus causing mutant p62 and its binding protein unable to be recruited into phagophores and interrupting the autophagy-mediated degradation pathways [219–221].Consequently, this leads to the accumulation of mutant SOD1 and TDP-43, further accelerating the development of ALS. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.