Recombinant Human Superoxide Dismutase 1 protein (ab84642)

Overview

Description

  • Nature
    Recombinant
  • Source
    Escherichia coli
  • Amino Acid Sequence
    • Species
      Human

Specifications

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.

  • Applications

    Western blot

    SDS-PAGE

  • Form
    Liquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    Preservative: None
    Constituents: 25% Glycerol, 50mM Tris HCl, 150mM Sodium chloride, 0.25mM DTT, 0.1mM PMSF, pH 7.5

General Info

  • Alternative names
    • 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
  • Function
    Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
  • Involvement in 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.
  • Sequence similarities
    Belongs to the Cu-Zn superoxide dismutase family.
  • Post-translational
    modifications
    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.
  • Cellular localization
    Cytoplasm. The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.
  • Information by UniProt

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References

ab84642 has not yet been referenced specifically in any publications.

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