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HomePediatrics: Genetics and Metabolic DiseaseSulfite Oxidase Deficiency and Molybdenum Cofactor Deficiency

Sulfite Oxidase Deficiency and Molybdenum Cofactor Deficiency

Background

Sulfite oxidase deficiency is an inborn error of the metabolism of sulfated amino acids. Individuals affected with sulfite oxidase deficiency most commonly present in the neonatal period with intractable seizures, characteristic dysmorphic features, and profound intellectual disability. Molybdenum cofactor deficiency, which affects the functioning of sulfite oxidase, leads to a similar phenotype.

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