Pathological intra-neuronal and intra-myocyte protein inclusions involving p62 are found in a variety of degenerative diseases such as amyotrophic lateral sclerosis (ALS) [7], frontotemporal lobar degeneration [3, 8], inclusion body myositis [9], Paget’s disease of bone [10, 11], and inclusion body myositis with Paget’s disease of bone and frontotemporal lobar degeneration [12]. Here, SQSTM1 is linked to amyotrophic lateral sclerosis.