Control Products
Product Info
*The optimal dilutions should be determined by the end user. For optimal experimental results, antibody reuse is not recommended.
*Tips:
WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.
Fold/Unfold
ALS; ALS1; Amyotrophic lateral sclerosis 1 adult; Cu/Zn SOD; Cu/Zn superoxide dismutase; Epididymis secretory protein Li 44; HEL S 44; 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;
Immunogens
A synthetic peptide from human SOD1
- P00441 SODC_HUMAN:
- Protein BLAST With
- NCBI/
- ExPASy/
- Uniprot
MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ
Research Backgrounds
Destroys radicals which are normally produced within the cells and which are toxic to biological systems.
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 responsible for the non-disulfide-linked homodimerization. Such modification might only occur in extreme conditions and additional experimental evidence is required.
Palmitoylation helps nuclear targeting and decreases catalytic activity.
Succinylation, adjacent to copper catalytic site, probably inhibits activity. Desuccinylation by SIRT5 enhances activity.
Cytoplasm. Mitochondrion. Nucleus.
Note: Predominantly cytoplasmic; the pathogenic variants ALS1 Arg-86 and Ala-94 gradually aggregates and accumulates in mitochondria.
Belongs to the Cu-Zn superoxide dismutase family.
Research Fields
· Cellular Processes > Transport and catabolism > Peroxisome. (View pathway)
· Human Diseases > Neurodegenerative diseases > Amyotrophic lateral sclerosis (ALS).
· Human Diseases > Neurodegenerative diseases > Huntington's disease.
· Human Diseases > Neurodegenerative diseases > Prion diseases.
· Organismal Systems > Aging > Longevity regulating pathway - multiple species. (View pathway)
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