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merck millipore,默克密理博,AB5480,Anti-Superoxide Dismutase 1 Antibody, aa 25-37
产品名称:Anti-Superoxide Dismutase 1 Antibody, aa 25-37
产品型号:AB5480
Anti-Superoxide Dismutase 1 Antibody, aa 25-37 detects level of Superoxide Dismutase 1 & has been published & validated for use in WB.
merck millipore,默克密理博,AB5480,Anti-Superoxide Dismutase 1 Antibody, aa 25-37
- 产品介绍
重要规格表
品种反应性 主要应用 宿主 格式 抗体类型 H WB Rb Serum Polyclonal Antibody 描述 产品目录编号 AB5480 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Superoxide Dismutase 1 Antibody, aa 25-37 Alternate Names - SOD1
背景信息 Mutations in the copper/ zinc superoxide dismutase (SOD1) gene are associated with 15- 20% of the familial forms of motoneuron disease. This 153 amino acid metalloenzyme is expressed in virtually all cells of all organisms above bacteria and is highly conserved across species, although some minor variations do occur. The incorporation of the mutated form of the SOD1 human gene into a transgenic mouse leads to the onset of the disease that closely resembles the human condition. These animals become weak at about 2-4 months of age and rapidly lose function, which results in death 4- 6 weeks later. 产品信息 格式 Serum 演示 Rabbit serum. Lyophilized. Contains no preservative. Reconstitute with 50 μL of sterile water. 应用 应用 Anti-Superoxide Dismutase 1 Antibody, aa 25-37 detects level of Superoxide Dismutase 1 & has been published & validated for use in WB. 主要应用 - Western Blotting
应用说明 Western blotting: 1:500
Optimal working dilutions must be determined by end user.生物信息 免疫原品种 Synthetic peptide corresponding to amino acids 25-37 of human SOD1. 表位 aa 25-37 宿主 Rabbit 特异性 Superoxide Dismutase 1 (SOD1), human specific. 品种反应性 Human 抗体类型 Polyclonal Antibody Entrez基因编号 - NM_000454.4
Entrez基因汇总 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. 基因符号 - EC 1.15.1.1
- SOD1
- ALS1
- IPOA
- Cu-Zn
- SOD
- ALS
- homodimer
非反应性品种 - Mouse
UniProt编号 - P00441
UniProt汇总 FUNCTION: SwissProt: P00441 # Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
COFACTOR: Binds 1 copper ion per subunit. & Binds 1 zinc ion per subunit.
SIZE: 154 amino acids; 15936 Da
SUBUNIT: Homodimer. The pathogenics variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not.
SUBCELLULAR LOCATION: Cytoplasm.
PTM: Unlike wild-type protein, the pathogenics variants ALS1 Arg- 38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A; which leads to their proteasomal degradation.
DISEASE: SwissProt: P00441 # 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.
SIMILARITY: SwissProt: P00441 ## Belongs to the Cu-Zn superoxide dismutase family.
MISCELLANEOUS: Zinc binding promotes dimerization.产品使用声明 使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Maintain lyophilized material at -70°C (dry) for up to 12 months. After reconstitution maintain frozen at -20°C in undiluted aliquots for up to 6 months. Avoid repeated freeze/thaw cycles. Glycerol (1:1) can be added for additional stability. 包装信息 数量 50 µL
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