Superoxide Dismutase, Cu,Zn (Superoxide Dismutase Cu/Zn, SOD Cu,Zn, Cu/Zn SOD, CuZnSOD, SODC, Amyotrophic Lateral Sclerosis 1, ALS1, ALS, Ipo1, Ipo-1, Superoxide Dismutase 1 (Soluble), SOD1, SOD-1)

FUNCTION: Destroys radicals which are normally produced within the cells and which are toxic to biological systems. CATALYTIC ACTIVITY: 2 superoxide + 2 H+ = O2 + H2O2. COFACTOR: Binds 1 copper ion per subunit. COFACTOR: Binds 1 zinc ion per subunit. SUBUNIT: Homodimer. SUBCELLULAR LOCATION: Cytoplasm. DISEASE: Defects in SOD1 are the cause of familial amyotrophic lateral sclerosis (FALS); also called amyotrophic lateral sclerosis 1 (ALS1 or ALS). ALS is a degenerative disorder of motorneurons in the cortex, brainstem and spinal cord. ALS is characterized by muscular weakness and atrophy beginning in the hands and spreading to the forearms and legs. Muscle fasciculations are commonly visible. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. ALS is sometimes referred to as Lou Gehrig disease after the famous American baseball player who was diagnosed with the disorder. FALS, the familial form of ALS, accounts for about 10% of the cases and is transmitted in an autosomal dominant manner. The mean age at onset of FALS is 45 years. MISCELLANEOUS: Zinc binding promotes dimerization. SIMILARITY: Belongs to the Cu-Zn superoxide dismutase family.\n\nApplications: \nSuitable for use in Western Blot. Other applications not tested. \n\nRecommended Dilution:\nWestern Blot: 1:500-1:1000\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nLyophilized powder may be stored at -20 degrees C. Stable for 12 months at -20 degrees C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Reconstituted product is stable for 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

S8060-01M

Size

100ul

Applications

WB

Hosts

Rabbit

Reactivities

Mouse, Rat

Form

Supplied as a lyophilized powder. Reconstitute with 100ul sterile dH2O.

P Type

Pab

Purity

Serum

Isotype

IgG

References

1. Jabusch J.R, et al. Biochemistry 19:2310-2316(1980), 2. Levanon D, et al. EMBO J. 4:77-84(1985), 3. Hallewell R.A, et al. Nucleic Acids Res. 13:2017-2034(1985), 4. Sherman L, et al. Proc. Natl. Acad. Sci. U.S.A. 80:5465-5469(1983), 5. Kajihara J, et al. J. Biochem. 104:851-854(1988), 6. Alexander M.D, et al. Ann. Neurol. 52:680-683(2002), 7. Gellera C, et al. Neuromuscul. Disord. 11:404-410(2001), 8. Murakami T, et al. J. Neurol. Sci. 189:45-47(2001), 9. Penco S, et al. Neurology 53:404-406(1999), 10. Morita M, et al. Neurosci. Lett. 205:79-82(1996), 11. Bartlett, et al. J. Neuroscience Methods. 98(1): 63-7(2000)

Additional Info

Recognizes mouse superoxide dismutase. Species Crossreactivity: rat.

Alternative Names

Superoxide Dismutase [Cu-Zn], B430204E11Rik, EC=1.15.1.1, MGC107553