Superoxide Dismutase, Cu,Zn (Superoxide Dismutase Cu/Zn, SOD Cu,Zn, Cu/Zn SOD, Amyotrophic Lateral Sclerosis 1, ALS1, ALS, Homodimer, Indophenoloxidase A, IPOA, Superoxide Dismutase 1, Superoxide Dismutase 1 Soluble, SOD1, SOD-1, Superoxide Dismutase Cyst

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. \nZinc binding promotes dimerization. Belongs to the Cu-Zn superoxide dismutase family.\n\nDefects 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. \n\nApplications: \nSuitable for use in Western Blot. Other applications not tested.\n\nRecommended Dilution:\nOptimal dilutions to be determined by the researcher.\n\nCellular Localization:\nCytoplasm\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile glycerol (40-50%), aliquot and store at -20 degrees C. Aliquots are stable for at least 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

S8060-01F

Size

100ul

Applications

WB

Hosts

Rabbit

Reactivities

Mouse, Rat

Form

Supplied as a lyophilized powder. Reconstitute in 100ul of sterile water. Centrifuge to remove any insoluble material.

P Type

Pab

Purity

Serum

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

Specificity was confirmed by western blot detecting mouse superoxide dismutase (SOD1). Species Crossreactivity: This antiserum is known to react with mouse and rat superoxide dismutase (SOD1) protein.