AFfirm™ SOD1 Mouse Monoclonal Antibody - #BF8509
製品説明
*The optimal dilutions should be determined by the end user.
*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.
折りたたみ／展開
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;
免疫原
- P00441 SODC_HUMAN:
- Protein BLAST With
- NCBI/
- ExPASy/
- Uniprot
MATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ
PTMs - P00441 基板として
| Site | PTM Type | Enzyme | Source |
|---|---|---|---|
| A2 | Acetylation | Uniprot | |
| T3 | Phosphorylation | Uniprot | |
| K4 | Ubiquitination | Uniprot | |
| C7 | S-Nitrosylation | Uniprot | |
| K10 | Acetylation | Uniprot | |
| K10 | Ubiquitination | Uniprot | |
| K24 | Acetylation | Uniprot | |
| K24 | Ubiquitination | Uniprot | |
| S26 | Phosphorylation | Uniprot | |
| K31 | Ubiquitination | Uniprot | |
| S35 | Phosphorylation | Uniprot | |
| K37 | Ubiquitination | Uniprot | |
| T40 | Phosphorylation | Uniprot | |
| T59 | Phosphorylation | Uniprot | |
| S60 | Phosphorylation | Uniprot | |
| S69 | Phosphorylation | Uniprot | |
| K71 | Acetylation | Uniprot | |
| K71 | Ubiquitination | Uniprot | |
| K76 | Sumoylation | Uniprot | |
| K76 | Ubiquitination | Uniprot | |
| R80 | Methylation | Uniprot | |
| T89 | Phosphorylation | Uniprot | |
| K92 | Ubiquitination | Uniprot | |
| S99 | Phosphorylation | Uniprot | |
| S103 | Phosphorylation | Uniprot | |
| S106 | Phosphorylation | Uniprot | |
| S108 | Phosphorylation | Uniprot | |
| K123 | Acetylation | Uniprot | |
| K123 | Ubiquitination | Uniprot | |
| K129 | Ubiquitination | Uniprot | |
| K137 | Ubiquitination | Uniprot | |
| R144 | Methylation | Uniprot | |
| C147 | S-Nitrosylation | Uniprot |
研究背景
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.
Homodimer; non-disulfide linked. Homodimerization may take place via the ditryptophan cross-link at Trp-33. The pathogenic variants ALS1 Arg-38, Arg-47, Arg-86 and Ala-94 interact with RNF19A, whereas wild-type protein does not. The pathogenic variants ALS1 Arg-86 and Ala-94 interact with MARCH5, whereas wild-type protein does not.
Belongs to the Cu-Zn superoxide dismutase family.
研究領域
· 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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