Destroys radicals which are normally produced within the cells and which are toxic to biological systems. Homodimer; non-disulfide linked. Homodimerization may take place via the ditryptophan cross-link at Trp-33. The pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not. The pathogenic variants ALS1 Arg-86 and Ala-94 interact with MARCH5, whereas wild-type protein does not. Belongs to the Cu-Zn superoxide dismutase family. Note: This description may include information from UniProtKB.
Protein type: Mitochondrial; Oxidoreductase; EC 18.104.22.168; Apoptosis
Molecular Function: identical protein binding; protein binding; copper ion binding; protein homodimerization activity; zinc ion binding; chaperone binding; superoxide dismutase activity; Rac GTPase binding; protein phosphatase 2B binding
Biological Process: positive regulation of catalytic activity; activation of MAPK activity; positive regulation of apoptosis; cellular iron ion homeostasis; myeloid cell homeostasis; retrograde axon cargo transport; muscle maintenance; retinal homeostasis; glutathione metabolic process; regulation of mitochondrial membrane potential; neurofilament cytoskeleton organization and biogenesis; positive regulation of superoxide release; negative regulation of neuron apoptosis; placenta development; response to drug; positive regulation of cytokine production; platelet activation; cell aging; transmission of nerve impulse; regulation of organ growth; response to ethanol; heart contraction; response to heat; superoxide release; cell death; relaxation of vascular smooth muscle; removal of superoxide radicals; locomotory behavior; response to organic substance; platelet degranulation; sensory perception of sound; ovarian follicle development; regulation of blood pressure; auditory receptor cell stereocilium organization and biogenesis; response to axon injury; negative regulation of cholesterol biosynthetic process; anterograde axon cargo transport; regulation of Rac GTPase activity; response to nutrient levels; response to superoxide; thymus development; regulation of T cell differentiation in the thymus; response to amphetamine; superoxide metabolic process; myelin maintenance in the peripheral nervous system; regulation of multicellular organism growth; response to copper ion; response to hydrogen peroxide; spermatogenesis; blood coagulation; regulation of protein kinase activity; hydrogen peroxide biosynthetic process; embryo implantation
SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.