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Target intelligence / Profile preview
Sulfite oxidase (SO)
- Target
- SO
- Molecular classification
- Enzyme, Molybdopterin-dependent mononuclear molybdoenzyme
01
Overview
Sulfite oxidase is a mitochondrial enzyme that catalyzes the oxidation of sulfite to sulfate, the terminal step in the catabolism of sulfur-containing amino acids. It plays a crucial role in detoxifying sulfite and maintaining sulfur amino acid metabolism. Mutations in the SUOX gene, encoding sulfite oxidase, can lead to severe metabolic and neurological disorders.
02
Biological functions
Oxidation of sulfite to sulfateSulfur amino acid catabolismDetoxification of sulfiteElectron transfer in the electron transport chain
03
Disease associations
Sulfite oxidase deficiencyMolybdenum cofactor deficiency
04
Safety considerations
Severe neurological impairment due to sulfite accumulationEarly onset brain atrophyPotential fatality if untreated
05
Biomarkers
Elevated urinary excretion of thiosulfateElevated urinary excretion of S-sulfocysteine
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