Mitochondrial oxidative metabolism seems to be involved in ALS pathogenesis, since morphological and functional alterations in mitochondria of spinal MNs and skeletal muscle of ALS patients have been described [3-6], as well as mitochondrial swelling and vacuolization in the spinal cord of transgenic mutant superoxide dismutase 1 (SOD1) ALS mice [7,8]. Here, SOD1 is linked to amyotrophic lateral sclerosis.