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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. Note: This description may include information from UniProtKB.
Protein type: Apoptosis; EC; Mitochondrial; Nuclear receptor co-regulator; Oxidoreductase
Chromosomal Location of Human Ortholog: 21q22.11
Cellular Component: cell soma; cytoplasm; cytoplasmic vesicle; cytosol; dendrite cytoplasm; extracellular matrix; extracellular region; extracellular space; mitochondrial intermembrane space; mitochondrion; nucleoplasm; nucleus; peroxisome; plasma membrane; protein complex
Molecular Function: chaperone binding; copper ion binding; identical protein binding; protein binding; protein phosphatase 2B binding; Rac GTPase binding; superoxide dismutase activity; zinc ion binding
Biological Process: activation of MAPK activity; anterograde axon cargo transport; auditory receptor cell stereocilium organization and biogenesis; cell aging; cellular iron ion homeostasis; embryo implantation; glutathione metabolic process; heart contraction; hydrogen peroxide biosynthetic process; locomotory behavior; muscle maintenance; myelin maintenance in the peripheral nervous system; myeloid cell homeostasis; negative regulation of cholesterol biosynthetic process; negative regulation of neuron apoptosis; neurofilament cytoskeleton organization and biogenesis; ovarian follicle development; platelet degranulation; positive regulation of apoptosis; positive regulation of catalytic activity; positive regulation of cytokine production; positive regulation of superoxide release; regulation of blood pressure; regulation of GTPase activity; regulation of mitochondrial membrane potential; regulation of multicellular organism growth; regulation of protein kinase activity; relaxation of vascular smooth muscle; removal of superoxide radicals; response to axon injury; response to drug; response to ethanol; response to heat; response to hydrogen peroxide; response to organic substance; response to superoxide; retinal homeostasis; retrograde axon cargo transport; sensory perception of sound; spermatogenesis; superoxide metabolic process; transmission of nerve impulse
Disease: Amyotrophic Lateral Sclerosis 1
Reference #:  P00441 (UniProtKB)
Alt. Names/Synonyms: ALS; ALS1; Cu/Zn superoxide dismutase; homodimer; indophenoloxidase A; IPOA; SOD; SOD, soluble; SOD1; SODC; superoxide dismutase; superoxide dismutase 1, soluble; Superoxide dismutase [Cu-Zn]; superoxide dismutase, cystolic
Gene Symbols: SOD1
Molecular weight: 15,936 Da
Basal Isoelectric point: 5.7  Predict pI for various phosphorylation states
Protein-Specific Antibodies or siRNAs from Cell Signaling Technology® Total Proteins
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