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Upingbio
SKU:YP-Ab-02339-10UL
SOD-1 Monoclonal Antibody
SOD-1 Monoclonal Antibody
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- Reaction species: Human;Mouse
- Gene Name: SOD1
- Protein name: Superoxide dismutase [Cu-Zn]
- Immunogen: Purified recombinant fragment of human SOD-1 expressed in E. Coli.
- Specificity: SOD-1 Monoclonal Antibody detects endogenous levels of SOD-1 protein.
- Composition: Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.
- Source: Monoclonal, Mouse
- Dilution ratio: Western Blot: 1/500 - 1/2000. Immunofluorescence: 1/200 - 1/1000. Flow cytometry: 1/200 - 1/400. ELISA: 1/10000. Not yet tested in other applications.
- Purification process: Affinity purification
- Storage: -20°C/1 year
- Other Names: SOD1; Superoxide dismutase [Cu-Zn]; Superoxide dismutase 1; hSod1
- Background: The protein encoded by this gene binds copper and zinc ions and is one of two isozymes responsible for destroying free superoxide radicals in the body. The encoded isozyme is a soluble cytoplasmic protein, acting as a homodimer to convert naturally-occuring but harmful superoxide radicals to molecular oxygen and hydrogen peroxide. The other isozyme is a mitochondrial protein. Mutations in this gene have been implicated as causes of familial amyotrophic lateral sclerosis. Rare transcript variants have been reported for this gene. [provided by RefSeq, Jul 2008],
- Function: catalytic activity:2 superoxide + 2 H(+) = O(2) + H(2)O(2).,cofactor:Binds 1 copper ion per subunit.,cofactor:Binds 1 zinc ion per subunit.,disease:Defects in SOD1 are the cause of amyotrophic lateral sclerosis type 1 (ALS1) [MIM:105400]. ALS1 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of cases leading to familial forms.,function:Destroys radicals which are normally produced within the cells and which are toxic to biological systems.,miscellaneous:The protein (both wild-type and ALS1 variants) has a tendency to form fibrillar aggregates in the
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