Interestingly, the absence of two autophagy-related proteins, Pab1-binding protein 1 (Pbp1) (orthologue of the human ALS risk-related gene coding for Ataxin-2 [100]) and Tip41, has been demonstrated to alleviate TDP-43 toxicity by counteracting its inhibition of autophagy [101,102]. Here, TARDBP is linked to amyotrophic lateral sclerosis.