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Superoxide Dismutase 1 protein (Tagged) (ab84642)

Overview

  • Product nameSuperoxide Dismutase 1 protein (Tagged)See all Superoxide Dismutase 1 proteins and peptides ...
  • Protein descriptionRecombinant full length Human Superoxide Dismutase 1 with N-terminal proprietary tag; amino acids 1-54, MW 45kDa. NM_000454. Swiss ID P00441
  • Expression hostE. coli
  • Properties

  • Purification notesPurity >90% as determined by densitometry.
  • FormLiquid
  • Storage instructionsShipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
  • Storage bufferPreservative: None
    Constituents: 25% Glycerol, 50mM Tris HCl, 150mM Sodium chloride, 0.25mM DTT, 0.1mM PMSF, pH 7.5
  • Concentration information loading...
  • Research Areas
  • Applications

    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.

    Application Notes
    WB WB: Use at an assay dependent dilution. Detects a band of approximately 45 kDa (predicted molecular weight: 45 kDa).
    SDS-PAGE SDS-PAGE: Use at an assay dependent dilution.

    Protein info

    • Alternative names
        ALSALS1Amyotrophic lateral sclerosis 1 adult
        Cu/Zn SODCu/Zn superoxide dismutaseHomodimerhSod1Indophenoloxidase AIPOAMn superoxide dismutaseSODSOD solubleSOD1SOD2SODCSODC_HUMANSuperoxide dismutase [Cu-Zn]Superoxide dismutase 1Superoxide dismutase 1 solubleSuperoxide dismutase Cu ZnSuperoxide dismutase cystolic
      see all
  • FunctionDestroys radicals which are normally produced within the cells and which are toxic to biological systems.
  • Involvement in diseaseDefects 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 similaritiesBelongs 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 localizationCytoplasm. The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.
  • Target information above from: UniProt accession P00441 The UniProt Consortium
    The Universal Protein Resource (UniProt) in 2010
    Nucleic Acids Res. 38:D142-D148 (2010) .

    Information by UniProt

    Superoxide Dismutase 1 protein (Tagged) images

    References for Superoxide Dismutase 1 protein (Tagged) (ab84642)

    ab84642 has not yet been referenced specifically in any publications.

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