Product Pathways - Neuroscience
SOD1 (71G8) Mouse mAb #4266
|W IP||H||Endogenous||18||Mouse IgG1|
Reactivity Key: H=Human
Species cross-reactivity is determined by western blot. Species enclosed in parentheses are predicted to react based on 100% sequence homology.
Specificity / Sensitivity
SOD1 (71G8) Mouse mAb detects endogenous levels of SOD1 protein. It does not cross-react with other related proteins.
Source / Purification
Monoclonal antibody is produced by immunizing animals with full-length recombinant human SOD1 protein.
SOD1, Cu/Zn superoxide dismutase, is a major antioxidant enzyme that catalyzes the conversion of superoxide anion to hydrogen peroxide and molecular oxygen (1). SOD1 is ubiquitously expressed and is localized in the cytosol, nucleus and mitochondrial intermembrane space. The SOD1 gene locus is on chromosome 21 in a region affected in Down Syndrome (2). In addition, over 100 distinct SOD1 inherited mutations have been identified in the familial form of amyotrophic lateral sclerosis (ALS), a progressive degenerative disease of motor neurons (3-5). Despite the fact that SOD1 helps to eliminate toxic reactive species, its mutations in ALS have been described as gain-of-function (5). The mechanism by which mutant SOD1 induces the neurodegeneration observed in ALS is still unclear. Mutant SOD1 proteins become misfolded and consequently oligomerize into high molecular weight species that aggregate and end up in proteinaceous inclusions (5).
- McCord, J.M. and Fridovich, I. (1988) Free Radic Biol Med 5, 363-9.
- Sherman, L. et al. (1983) Proc Natl Acad Sci USA 80, 5465-9.
- Rosen, D.R. et al. (1993) Nature 362, 59-62.
- Deng, H.X. et al. (1993) Science 261, 1047-51.
- Valentine, J.S. and Hart, P.J. (2003) Proc Natl Acad Sci USA 100, 3617-22.
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For Research Use Only. Not For Use In Diagnostic Procedures.