Recombinant human Superoxide Dismutase 1 protein (ab201408)
- Datasheet
- References
- Protocols
Overview
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Product name
Recombinant human Superoxide Dismutase 1 protein
See all Superoxide Dismutase 1 proteins and peptides -
Protein length
Full length protein
Description
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Nature
Recombinant -
Source
Escherichia coli -
Amino Acid Sequence
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Accession
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Species
Human -
Sequence
MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHE FGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSI EDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVI GIAQ -
Molecular weight
31 kDa -
Amino acids
1 to 154 -
Additional sequence information
Homodimer, non-glycosylated polypeptide chain containing 2 x 154 amino acids.
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Associated products
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Related Products
Specifications
Our Abpromise guarantee covers the use of ab201408 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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Biological activity
Fully biologically active when compared to standard.
The potency per mg was tested by Pyrogallic Acid method and was found to be more than 10,000Units/mg.
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Applications
Functional Studies
HPLC
SDS-PAGE
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Endotoxin level
< 1.000 Eu/µg -
Purity
> 95 % SDS-PAGE.
>95% by HPLC analysis. -
Form
Lyophilised -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped at 4°C. Store at -20°C long term. Avoid freeze / thaw cycle.
pH: 7.40
Constituent: 100% PBS
Lyophilised from a 0.2µm filtered solution.This product is an active protein and may elicit a biological response in vivo, handle with caution.
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ReconstitutionBriefly centrifuge the vial prior to opening to bring the contents to the bottom. Reconstitute in sterile distilled water or aqueous buffer containing 0.1% BSA to a concentration of 0.1-1.0 mg/ml. Stock solutions should be apportioned into working aliquots and stored at -20°C to -70°C. Further dilutions should be made in appropriate buffered solutions.
General Info
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Alternative names
- ALS
- ALS1
- Amyotrophic lateral sclerosis 1 adult
see all -
Function
Destroys radicals which are normally produced within the cells and which are toxic to biological systems. -
Involvement in disease
Defects 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 similarities
Belongs to the Cu-Zn superoxide dismutase family. -
Post-translational
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 localization
Cytoplasm. The pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria. - Information by UniProt
Datasheets and documents
References
ab201408 has not yet been referenced specifically in any publications.
