What causes sulfite oxidase deficiency?

What causes sulfite oxidase deficiency?

Isolated sulfite oxidase deficiency is caused by a mutation in the SUOX gene (12q13. 13) (see this term). The SUOX gene encodes the enzyme sulfite oxidase which catalyzes sulfite to sulfate, a process essential for the catabolism of sulfur-containing amino acids.

Which foods contain molybdenum?

What foods provide molybdenum?

• Legumes such as black-eyed peas and lima beans.
• Whole grains, rice, nuts, potatoes, bananas, and leafy vegetables.
• Dairy products, like milk, yogurt, and cheese.
• Beef, chicken, and eggs.

Can you test for sulfite oxidase?

Molecular testing of the known genes is available for diagnosis confirmation. However, in the acute phase, a diagnosis of sulfite oxidase deficiency is generally based on the presence or absence of physical findings and characteristic metabolites.

Does sulfite oxidase contain molybdenum?

Sulfite oxidase contains both molybdenum cofactor and heme as prosthetic groups, and uses cytochrome c as an electron acceptor.

How do you fix molybdenum deficiency?

Molybdenum is more likely to be deficient in acid soils. Liming to bring pH above 5.5 usually fixes molybdenum deficiency in the long term. Applying a molybdenum fertiliser can correct molybdenum deficiency symptoms in plants more quickly.

What happens if you have too little molybdenum?

Molybdenum deficiency is very rare in the United States. It happens only in people with a very rare genetic disorder called molybdenum cofactor deficiency. This disorder prevents the body from using molybdenum. It can cause seizures and severe brain damage that usually leads to death within days after birth.

What is the best food source of molybdenum?

The top sources of molybdenum in U.S. diets are legumes, cereal grains, leafy vegetables, beef liver, and milk [17]. Milk and cheese products are the main sources of molybdenum for teens and children [19].

What happens when molybdenum cofactor is deficient?

Sulfite oxidase deficiency and molybdenum cofactor deficiency in the metabolism of sulfated amino acids. When sulfite oxidase is deficient, alternate metabolic pathways for sulfite are augmented, including formation of metabolites s-sulfocysteine and thiosulfate. S-sulfocysteine probably substitutes for cysteine in connective tissues.

Which is the enzyme associated with molybdenum deficiency?

However, most cases of this disorder are associated with deficiency of the molybdenum-containing pterin cofactor (molybdenum cofactor deficiency). Molybdenum cofactor is associated with the enzymes sulfite oxidase, xanthine dehydrogenase, and aldehyde oxidase (see the image below).

How does autosomal recessive inheritance work in molybdenum deficiency?

Molybdenum cofactor deficiency has an autosomal recessive pattern of inheritance, which means both copies of the gene in each cell have mutations. An affected individual usually inherits one altered copy of the gene from each parent.