Function
Catalyzes the oxidation of sulfite to sulfate, the terminal reaction in the oxidative degradation of sulfur-containing amino acids. Can run in reverse direction and reduce nitrite to nitric oxide under physiologically relevant conditions, such as hypoxia.
Biological Context
Subcellular Location: Mitochondrion intermembrane space
Disease Association: Sulfite oxidase deficiency, isolated (ISOD) : A life-threatening, autosomal recessive neurometabolic disorder characterized by severe neurological impairment. Classic ISOD manifests in the first few hours to days of life and is characterized by intractable seizures, feeding difficulties, rapidly progressive encephalopathy, microcephaly, and profound intellectual disability. Children usually die during the first few months of life. Mild ISOD manifests in infancy or early childhood and is characterized by ectopia lentis that is variably present, developmental delay and regression, movement disorder characterized by dystonia and choreoathetosis, ataxia, and rarely acute hemiplegia due to metabolic stroke. [The disease is caused by variants affecting the gene represented in this entry]
Pathway: Energy metabolism; sulfur metabolism
Product Specifications
Recombinant Human Sulfite oxidase, mitochondrial (SUOX) is a recombinant protein from Homo sapiens (Human), expressed in E.coli, covering amino acids 80-545aa, with N-terminal 6xHis-SUMO-tagged tag, molecular weight 67.6kDa, purity Greater than 90% as determined by SDS-PAGE.. Suitable for ELISA and Western Blot applications. Explore more Enzyme proteins →
