| Background | This ELISA kit uses the Competitive-ELISA principle. The micro ELISA plate provided in this kit has been pre-coated with Human SOD1. During the reaction, Human SOD1 in the sample or standard competes with a fixed amount of Human SOD1 on the solid phase supporter for sites on the Biotinylated Detection Ab specific to Human SOD1. Excess conjugate and unbound sample or standard are washed from the plate, and Avidin conjugated to Horseradish Peroxidase (HRP) are added to each microplate well and incubated. Then a TMB substrate solution is added to each well. The enzyme-substrate reaction is terminated by the addition of stop solution and the color change is measured spectrophotometrically at a wavelength of 450 nm ± 2 nm. The concentration of Human SOD1 in the samples is then determined by comparing the OD of the samples to the standard curve. |
| 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. |
| Database Links | Reactome: R-HSA-114608 |
| Cellular Localisation | CytoplasmMitochondrionNucleusPredominantly CytoplasmicThe Pathogenic Variants Als1 Arg-86 And Ala-94 Gradually Aggregates And Accumulates In Mitochondria |
| Protein Name | Superoxide Dismutase Cu-ZnSuperoxide Dismutase 1Hsod1 |
| Alternative Names | Anti-Superoxide Dismutase Cu-Zn antibodyAnti-Superoxide Dismutase 1 antibodyAnti-Hsod1 antibodyAnti-SOD1 antibody |
Information sourced from Uniprot.org
