Sulfite oxidase

The sulphite oxidase is an enzyme that is found in most living organisms, and which catalyzes the conversion of sulfite to sulfate. It is localized in the mitochondria of all eukaryotic cells. Also in many bacterial species is a related sulfite encountered. In mammals, the sulfite oxidase occurs mainly in the liver, kidneys, spleen, lungs, brain and heart.

Function

70-95 % of the absorbed sulfite normally enters the bloodstream. Where it is oxidised to sulphate by the sulfite and transports the electrons cytochrome c ( electron transport chain ) with the aim of ATP production:

The sulfates formed are excreted in the urine.

Sulfidoxidase deficiency leads to drug-resistant neonatal seizures ( epilepsy) and a severe encephalopathy. Cause of a defect is either an isolated enzyme defect or a molybdenum cofactor deficiency, which can not be the clinical pictures differ.

  • The isolated enzyme defect:

The oxidation of sulfite to sulfate is disturbed, resulting in an increase of the sulfite concentration in all body fluids result.

  • Molybdenum cofactor deficiency ( see molybdenum cofactor deficiency ):

The synthesis of the molybdenum cofactor - or molybdopterin is disturbed, causing the enzymes sulfite oxidase, xanthine oxidase and aldehyde oxidase are limited in their activity. As a treatment option results in the molybdate supplementation.

Human Genome Organisation Hydroxylation
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