Product nameRecombinant Human Superoxide Dismutase 1 protein
See all Superoxide Dismutase 1 proteins and peptides
Purity> 95 % SDS-PAGE.
Expression systemEscherichia coli
Protein lengthFull length protein
- Anti-Superoxide Dismutase 1 antibody (ab13498)
- Anti-Superoxide Dismutase 1 antibody (ab13499)
- Anti-Superoxide Dismutase 1 antibody (ab16831)
- Anti-Superoxide Dismutase 1 antibody (ab45777)
- Anti-Superoxide Dismutase 1 antibody [EP1727Y] (ab51254)
- Anti-Superoxide Dismutase 1 antibody (ab52950)
- Anti-Superoxide Dismutase 1 antibody [EPR1726] (ab79390)
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.
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Preparation and Storage
Stability and Storage
Shipped at 4°C. Upon delivery aliquot. Store at -80°C. Avoid freeze / thaw cycle.
Constituent: 0.476% HEPES
- Amyotrophic lateral sclerosis 1 adult
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.
modificationsUnlike 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.
- Information by UniProt
ab82649 has not yet been referenced specifically in any publications.