Human Superoxide Dismutase 1 full length protein (ab82649)

Overview

Description

  • NatureRecombinant
  • SourceE. coli
  • Amino Acid Sequence
    • SpeciesHuman
    • Amino acids0 to 0

Specifications

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.

  • Applications

    SDS-PAGE

    Western blot

  • Purity> 95 % by SDS-PAGE.

  • FormLyophilised
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Aliquot and store at -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: None
    Constituents: 20mM HEPES, pH 7.4

General info

  • Alternative names
    • ALS
    • ALS1
    • Amyotrophic lateral sclerosis 1 adult
    • Cu/Zn SOD
    • Cu/Zn superoxide dismutase
    • 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
  • 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

Human Superoxide Dismutase 1 full length protein images

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

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

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

ab82649 has not yet been referenced specifically in any publications.

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