These ALS/FTD-linked mutations cause the accumulation of microtubule-associated protein 1 light chain 3 (LC3) and sequestosome 1 (SQSTM1)/p62, suggesting that malfunction of the autophagic pathway play a key role in the pathogenesis of neurodegeneration [7,8,9,16,17,18]. Here, SQSTM1 is linked to frontotemporal dementia.