• Target：
• SOD-1

• Fields：
• >>Peroxisome;>>Longevity regulating pathway - multiple species;>>Parkinson disease;>>Amyotrophic lateral sclerosis;>>Huntington disease;>>Prion disease;>>Pathways of neurodegeneration - multiple diseases;>>Chemical carcinogenesis - reactive oxygen species

• Gene Name：
• SOD1

• Protein Name：
• Superoxide dismutase [Cu-Zn]

• Human Gene Id：
• 6647

• Human Swiss Prot No：
• P00441

• Mouse Gene Id：
• 20655

• Mouse Swiss Prot No：
• P08228

• Rat Gene Id：
• 24786

• Rat Swiss Prot No：
• P07632

• Specificity：
• endogenous

• Formulation：
• PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA

• Source：
• Monoclonal, rabbit, IgG, Kappa

• Dilution：
• IHC 1:100-1:5000;WB 1:2000-1:10000;IF 1:200-1:1000;ELISA 1:5000-1:20000;IP 1:50-1:200;

• Purification：
• Protein A

• Storage Stability：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• Other Name：
• SOD1;Superoxide dismutase [Cu-Zn];Superoxide dismutase 1;hSod1

• Molecular Weight(Da)：
• 23kD

• Observed Band(KD)：
• 15kD

• 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

• Subcellular Location：
• Cytoplasm, Nucleus

• Expression：
• Colon,Fetal brain cortex,Placenta,

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