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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.
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| Protein type: Apoptosis; Oxidoreductase; EC 1.15.1.1; Mitochondrial |
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Cellular Component: dendrite cytoplasm; extracellular space; protein complex; mitochondrion; extracellular region; cytosol; extracellular matrix; cell soma; mitochondrial matrix; cytoplasm; nucleolus; plasma membrane; peroxisome; cytoplasmic vesicle; nucleus
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Molecular Function: protein binding; copper ion binding; protein homodimerization activity; zinc ion binding; chaperone binding; superoxide dismutase activity; protein phosphatase 2B binding
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Biological Process: positive regulation of catalytic activity; cellular iron ion homeostasis; positive regulation of apoptosis; activation of MAPK activity; myeloid cell homeostasis; retrograde axon cargo transport; muscle maintenance; retinal homeostasis; glutathione metabolic process; regulation of mitochondrial membrane potential; neurofilament cytoskeleton organization and biogenesis; negative regulation of neuron apoptosis; placenta development; response to drug; positive regulation of cytokine production; platelet activation; cell aging; regulation of organ growth; transmission of nerve impulse; response to ethanol; heart contraction; response to heat; superoxide release; relaxation of vascular smooth muscle; removal of superoxide radicals; locomotory behavior; response to organic substance; sensory perception of sound; platelet degranulation; ovarian follicle development; regulation of blood pressure; double-strand break repair; DNA fragmentation during apoptosis; auditory receptor cell stereocilium organization and biogenesis; response to axon injury; negative regulation of cholesterol biosynthetic process; anterograde axon cargo transport; 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; embryo implantation; hydrogen peroxide biosynthetic process
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Reference #:
P00441 (UniProtKB)
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| 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 |
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Molecular weight: 15,936 Da
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Basal Isoelectric point: 5.7
Predict pI for various phosphorylation states
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Protein-Specific Antibodies or siRNAs from Cell Signaling Technology®
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