Description
Product Usage Information
Western Blotting: 1:1000
Immunohistochemistry (Paraffin): 1:200 - 1:800
Storage
Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody. For a carrier-free (BSA and azide free) version of this product see product # 65778 .
Protocol
Available protocols: Western Blotting, Immunohistochemistry (Paraffin)
Specificity / Sensitivity
SOD1 (E4G1H) Rabbit Monoclonal Antibody recognizes endogenous levels of total SOD1 protein.
Species Reactivity: Human, Mouse, Rat
Source / Purification
Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues near the amino terminus of human SOD1 protein.
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).
Alternate Names
ALS; ALS1; Cu/Zn superoxide dismutase; epididymis secretory protein Li 44; HEL-S-44; homodimer; hSod1; indophenoloxidase A; IPOA; SOD; SOD, soluble; SOD1; SODC; STAHP; superoxide dismutase; Superoxide dismutase [Cu-Zn]; Superoxide dismutase 1; superoxide dismutase 1, soluble; superoxide dismutase, cystolic
