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It was demonstrated that in the spinal cord of the SOD1-G93A ALS mouse model, the mitochondrial receptor SQSTM1/p62 is recruited to the mitochondrial surface, where it activates mitophagy, whereas knockdown of the Parkin gene reduces the loss of motor neurons, thereby delaying the progression of disease in SOD1-G93A mice (Palomo et al., 2018)