| Post Translational Modifications | 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. |
| Function | Destroys radicals which are normally produced within the cells and which are toxic to biological systems. |
| Protein Name | Superoxide Dismutase Cu-ZnSuperoxide Dismutase 1Hsod1 |
| Database Links | Reactome: R-HSA-114608Reactome: R-HSA-3299685Reactome: R-HSA-8950505 |
| Cellular Localisation | CytoplasmNucleusPredominantly CytoplasmicThe Pathogenic Variants Als1 Arg-86 And Ala-94 Gradually Aggregates And Accumulates In Mitochondria |
| Alternative Antibody Names | Anti-Superoxide Dismutase Cu-Zn antibodyAnti-Superoxide Dismutase 1 antibodyAnti-Hsod1 antibodyAnti-SOD1 antibody |
Information sourced from Uniprot.org
