UCP1 and amyotrophic lateral sclerosis: Mice overexpressing uncoupling protein-1 (UCP1) in skeletal muscle have decreased mitochondrial membrane potential and respiratory control ratio (Dupuis et al., 2009) that is associated with muscle atrophy, a shift from a fast to slow muscle fiber type phenotype and a deterioration in the NMJ; alterations also observed in ALS mice (Gordon et al., 2010).