Importantly, autophagy was found altered in ALS muscles, with in particular, abnormal expressions of several autophagic markers, including LC3B and P62, depicted in the skeletal muscle of ALS mice (Dobrowolny et al., 2008; Crippa et al., 2013) or ALS-mimicking muscle cell cultures (Onesto et al., 2011). Here, SQSTM1 is linked to amyotrophic lateral sclerosis.