免疫原
Synthesized peptide derived from the Internal region of human SOD-1.
特异性
The antibody detects endogenous SOD1 protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
PBS, pH 7.4, containing 0.5%BSA, 0.02% sodium azide as Preservative and 50% Glycerol.
稀释比例
WB: 1/500 - 1/2000.IHC-p:1:50-300.. IF 1:50-200
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（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],
功能
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
蛋白名称
Superoxide dismutase [Cu-Zn]
其他名称
SOD1; Superoxide dismutase [Cu-Zn]; Superoxide dismutase 1; hSod1
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
细胞定位
Cytoplasm . Mitochondrion . Nucleus . Predominantly cytoplasmic; the pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria. .
组织表达
Colon,Fetal brain cortex,Placenta,
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