Energy metabolism and redox state are strictly linked; energy metabolism is a 
source of reactive oxygen species (ROS) that, in turn, regulate the flux of 
metabolic pathways. Moreover, to assure redox homeostasis, metabolic pathways 
and antioxidant systems are often coordinately regulated. Several findings show 
that superoxide dismutase 1 (SOD1) enzyme has effects that go beyond its 
superoxide dismutase activity and that its functions are not limited to the 
intracellular compartment. Indeed, SOD1 is secreted through unconventional 
secretory pathways, carries out paracrine functions and circulates in the blood 
bound to lipoproteins. Striking experimental evidence links SOD1 to the redox 
regulation of metabolism. Important clues are provided by the systemic effects 
on energy metabolism observed in mutant SOD1-mediated amyotrophic lateral 
sclerosis (ALS). The purpose of this review is to analyze in detail the 
involvement of SOD1 in redox regulation of metabolism, nutrient sensing, 
cholesterol metabolism and regulation of mitochondrial respiration. The 
scientific literature on the relationship between ALS, mutated SOD1 and 
metabolism will also be explored, in order to highlight the metabolic functions 
of SOD1 whose biological role still presents numerous unexplored aspects that 
deserve further investigation.