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Superoxide Dismutase 1 protein (Human) (ab82649)

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Overview

  • Product nameSuperoxide Dismutase 1 protein (Human)See all Superoxide Dismutase 1 proteins and peptides ...
  • Protein descriptionRecombinant full length Human Superoxide Dismutase 1, amino acids 1-154, MWt 16 kDa (Swiss-Prot P00441).
  • Expression hostE. coli

    • Properties

  • Purity> 95 % by SDS-PAGE
  • FormLyophilised
  • Storage instructionsAliquot and store at -80°C. Avoid repeated freeze / thaw cycles.
  • Storage bufferPreservative: None
    Constituents: 20mM HEPES, pH 7.4
  • Concentration information loading...
  • Research Areas

    • Applications

    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.

    Application Notes
    SDS-PAGE SDS-PAGE: Use at an assay dependent dilution.
    WB WB: Use at an assay dependent concentration. Ab82649 can be used as a WB positive control in conjunction with ab88404.

    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 (Human) images

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

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

      Secondary
      Goat polyclonal Secondary Antibody to Rabbit IgG - H&L (HRP), pre-adsorbed (ab97080) at 1/5000 dilution
      developed using the ECL technique

      Performed under reducing conditions.

      Exposure time : 30 seconds

    References for Superoxide Dismutase 1 protein (Human) (ab82649)

    ab82649 has not yet been referenced specifically in any publications.

    Publishing research using ab82649 ? Please let us know so that we can cite the reference in this datasheet

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    Dear AbCam Staff
    I am conducting some studies on SOD1 protein espression using human samples from ALS patiens.

    Since my next pilot projectwill be focused onPost Translational Modification of SOD1 I would like considering the following prod...

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    Thank you for your enquiry which has been forwarded to the scientific support team (mailto:technical@abcam.com).

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