重组人Superoxide Dismutase 1蛋白(ab84642)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% Densitometry
- Suitable for: WB, SDS-PAGE
描述
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产品名称
重组人Superoxide Dismutase 1蛋白
参阅全部 Superoxide Dismutase 1 蛋白酶 -
纯度
> 90 % Densitometry. -
表达系统
Escherichia coli -
蛋白长度
Full length protein -
无动物成分
No -
性质
Recombinant -
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种属
Human
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相关产品
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Related Products
技术指标
Our Abpromise guarantee covers the use of ab84642 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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应用
Western blot
SDS-PAGE
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形式
Liquid -
Concentration information loading...
制备和贮存
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稳定性和存储
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 7.50
Constituents: 0.00174% PMSF, 0.00385% DTT, 0.79% Tris HCl, 25% Glycerol (glycerin, glycerine), 0.87% Sodium chloride
常规信息
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别名
- ALS
- ALS1
- Amyotrophic lateral sclerosis 1 adult
see all -
功能
Destroys radicals which are normally produced within the cells and which are toxic to biological systems. -
疾病相关
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. -
序列相似性
Belongs to the Cu-Zn superoxide dismutase family. -
翻译后修饰
Unlike wild-type protein, the pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 are polyubiquitinated by RNF19A leading to their proteasomal degradation. The pathogenic variants ALS1 Arg-86 and Ala-94 are ubiquitinated by MARCH5 leading to their proteasomal degradation.
The ditryptophan cross-link at Trp-33 is reponsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required. -
细胞定位
Cytoplasm. The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria. - Information by UniProt
实验方案
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
数据表及文件
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SDS download
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Datasheet download
文献 (0)
ab84642 尚未被引用在任何文献中。