Recombinant人Superoxide Dismutase 1 protein (ab82649)

概述

描述

  • 性质Recombinant
  • 来源Escherichia coli
  • 氨基酸序列
    • 种属Human

技术指标

Our Abpromise guarantee covers the use of ab82649 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 应用

    SDS-PAGE

    Western blot

  • 纯度> 95 % SDS-PAGE.

  • 形式Lyophilised
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped at 4°C. Upon delivery aliquot. Store at -80°C. Avoid freeze / thaw cycle.

    Preservative: None
    Constituents: 20mM HEPES, pH 7.4

常规信息

  • 别名
    • ALS
    • ALS1
    • Amyotrophic lateral sclerosis 1 adult
    • Cu/Zn SOD
    • Cu/Zn superoxide dismutase
    • Epididymis secretory protein Li 44
    • HEL S 44
    • Homodimer
    • hSod1
    • Indophenoloxidase A
    • IPOA
    • Mn superoxide dismutase
    • SOD
    • SOD soluble
    • SOD1
    • SOD2
    • SODC
    • SODC_HUMAN
    • Superoxide dismutase [Cu-Zn]
    • Superoxide dismutase 1
    • Superoxide dismutase 1 soluble
    • Superoxide dismutase Cu Zn
    • Superoxide dismutase cystolic
    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

Recombinant Human Superoxide Dismutase 1 protein 图像

  • ab82649 (1µg) on SDS-PAGE.
  • All lanes : Anti-Superoxide Dismutase 1 antibody (ab88404) at 1 µg/ml

    Lane 1 : Recombinant Human Superoxide Dismutase 1 protein (ab82649) at 0.01 µg
    Lane 2 : Recombinant Human Superoxide Dismutase 1 protein (ab82649) at 0.001 µg

    Secondary
    Goat Anti-Rabbit IgG H&L (HRP) preadsorbed (ab97080) at 1/5000 dilution
    Developed using the ECL technique

    Performed under reducing conditions.

    Exposure time : 30 seconds

Recombinant Human Superoxide Dismutase 1 protein (ab82649)参考文献

ab82649 has not yet been referenced specifically in any publications.

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