Function
Destroys radicals which are normally produced within the cells and which are toxic to biological systems. Catalyzes the oxidation of hydrogen sulfide (H2S) to sulfate, playing an important role in detoxifying H2S and limiting the accumulation of reactive sulfur species (RSS) such as persulfides and polysulfides.
Biological Context
Subcellular Location: Cytoplasm; Nucleus
Disease Association: Amyotrophic lateral sclerosis 1 (ALS1) : A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. 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 the cases. [The disease is caused by variants affecting the gene represented in this entry] | Spastic tetraplegia and axial hypotonia, progressive (STAHP) : An autosomal recessive, neurologic disorder characterized by loss of motor abilities in the first year of life, after which severe, progressive spastic tetraparesis develops. Affected individuals have severe axial hypotonia, hyperekplexia, hypertonia, and myokymia, reflecting upper motor neuron involvement. Cognitive development may be affected. [The disease is caused by variants affecting the gene represented in this entry]
Product Specifications
Recombinant Human Superoxide dismutase [Cu-Zn] (SOD1) (Active) is a recombinant protein from Homo sapiens (Human), expressed in E.Coli, covering amino acids 2-154aa, with N-terminal 10xHis-tagged tag, molecular weight 20 kDa, purity >95% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications.
![Recombinant Human Superoxide dismutase [Cu-Zn] (SOD1) (Active), Homo sapiens (Human), E.Coli, N-terminal 10xHis-tagged, 20 kDa, purity >>95% as determined by SDS-PAGE.](https://www.biocrestsci.com/wp-content/uploads/protein/1/BCACP-000850-SDS.jpg)