During autophagy, the recognition of the lipidated form of microtubule‐associated protein light chain 3 isoform B (LC3B‐II) within the phagophore membrane by p62/SQSTM1 represents a critical protein–protein interaction that is mediated through its LC3‐interacting region, and notably, some FTLD and ALS mutations of p62/SQSTM1 map to this region, leading to reduction in LC3B binding affinity and limiting the recruitment of p62/SQSTM1 to the phagophore 80. This evidence concerns the gene SQSTM1 and amyotrophic lateral sclerosis.