Key features and details
- Rabbit polyclonal to Superoxide Dismutase 1 (Biotin)
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
- Conjugation: Biotin
- Isotype: IgG
Product nameAnti-Superoxide Dismutase 1 antibody (Biotin)
See all Superoxide Dismutase 1 primary antibodies
DescriptionRabbit polyclonal to Superoxide Dismutase 1 (Biotin)
Tested applicationsSuitable for: WBmore details
Species reactivityReacts with: Mouse, Rat, Human
Full length native protein (purified) corresponding to Superoxide Dismutase 1.
Database link: P00441
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Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle. Store In the Dark.
Storage bufferPreservative: 0.09% Sodium azide
Constituents: 50% Glycerol, 49% PBS
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab195505 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000. Predicted molecular weight: 16 kDa.|
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
- ALS antibody
- ALS1 antibody
- Amyotrophic lateral sclerosis 1 adult antibody
ab195505 has not yet been referenced specifically in any publications.