Human Superoxide Dismutase 1 full length protein (ab82649)
- Product nameHuman Superoxide Dismutase 1 full length proteinSee all Superoxide Dismutase 1 proteins and peptides ...
- SourceE. coli
- Amino Acid Sequence
- Amino acids0 to 0
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.
- Purity> 95
% by SDS-PAGE.
- Concentration information loading...
Preparation and Storage
- Stability and Storage
Aliquot and store at -80°C. Avoid repeated freeze / thaw cycles.
Constituents: 20mM HEPES, pH 7.4
- Amyotrophic lateral sclerosis 1 adult
- Cu/Zn SOD
- Cu/Zn superoxide dismutase
- Indophenoloxidase A
- Mn superoxide dismutase
- SOD soluble
- Superoxide dismutase [Cu-Zn]
- Superoxide dismutase 1
- Superoxide dismutase 1 soluble
- Superoxide dismutase Cu Zn
- Superoxide dismutase cystolic
- 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.
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
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 Human Superoxide Dismutase 1 full length protein (ab82649)
ab82649 has not yet been referenced specifically in any publications.