Protocols

4266:

Western Blotting,  Immunoprecipitation (Magnetic)

Background

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).

1. McCord, J.M. and Fridovich, I. (1988) Free Radic Biol Med 5, 363-9.
2. Sherman, L. et al. (1983) Proc Natl Acad Sci USA 80, 5465-9.
3. Rosen, D.R. et al. (1993) Nature 362, 59-62.
4. Deng, H.X. et al. (1993) Science 261, 1047-51.
5. Valentine, J.S. and Hart, P.J. (2003) Proc Natl Acad Sci USA 100, 3617-22.

Application References

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• 7076  Anti-mouse IgG, HRP-linked Antibody
• 7727  Biotinylated Protein Ladder Detection Pack
• 7072  Phototope-HRP Western Blot Detection System, Anti-mouse IgG, HRP-linked Antibody
• 2770  SOD1 Antibody
• 6883  SignalFire™ ECL Reagent
• 12757  SignalFire™ Elite ECL Reagent
• 12630  SignalFire™ Plus ECL Reagent

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