SOD1: 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.
Protein type: Mitochondrial; EC 1.15.1.1; Oxidoreductase; Nuclear receptor co-regulator; Apoptosis
Cellular Component: dendrite cytoplasm; extracellular space; neuron projection; protein complex; mitochondrion; cell; extracellular region; mitochondrial intermembrane space; cytosol; secretory granule; nucleoplasm; extracellular matrix; cell soma; cytoplasm; plasma membrane; peroxisome; intracellular; cytoplasmic vesicle; nucleus; myelin sheath
Molecular Function: identical protein binding; enzyme binding; copper ion binding; 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; cellular iron ion homeostasis; myeloid cell homeostasis; retrograde axon cargo transport; response to antibiotic; muscle maintenance; retinal homeostasis; glutathione metabolic process; neurofilament cytoskeleton organization and biogenesis; regulation of mitochondrial membrane potential; positive regulation of superoxide release; negative regulation of neuron apoptosis; response to drug; positive regulation of cytokine production; cell aging; transmission of nerve impulse; response to reactive oxygen species; response to ethanol; response to heat; heart contraction; response to oxidative stress; superoxide release; negative regulation of apoptosis; relaxation of vascular smooth muscle; removal of superoxide radicals; locomotory behavior; response to organic substance; sensory perception of sound; ovarian follicle development; double-strand break repair; regulation of blood pressure; DNA fragmentation during apoptosis; auditory receptor cell stereocilium organization and biogenesis; response to axon injury; anterograde axon cargo transport; negative regulation of cholesterol biosynthetic process; aging; response to nutrient levels; response to superoxide; 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; regulation of protein kinase activity; hydrogen peroxide biosynthetic process; embryo implantation
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