What is isolated sulfite oxidase deficiency?
Isolated sulfite oxidase deficiency (ISOD) is a disorder of the nervous system, with a severe “classic” form that starts in the newborn period and a milder, late-onset form that begins later in infancy or early childhood.
Is molybdenum a cofactor?
The proteins produced from each of these genes are involved in the formation (biosynthesis) of a molecule called molybdenum cofactor. Molybdenum cofactor, which contains the element molybdenum, is essential to the function of several enzymes.
What does sulfite oxidase do in the body?
Sulfite oxidase (EC 1.8. 3.1) is an enzyme in the mitochondria of all eukaryotes, with exception of the yeasts. It oxidizes sulfite to sulfate and, via cytochrome c, transfers the electrons produced to the electron transport chain, allowing generation of ATP in oxidative phosphorylation.
Is sulfite oxidase deficiency genetic?
Isolated sulfite oxidase deficiency (ISOD) is inherited in an autosomal recessive manner.
What are the symptoms of molybdenum deficiency?
The deficiency caused intellectual disability, seizures, opisthotonus, and lens dislocation. Molybdenum deficiency resulting in sulfite toxicity occurred in a patient receiving long-term total parenteral nutrition. Symptoms were tachycardia, tachypnea, headache, nausea, vomiting, and coma.
Can sulfites cause seizures?
The toxic effects of sulfites can trigger allergic reactions. Symptoms of sulfite sensitivity include asthma, hives, tissue swelling, abdominal pain, nausea, diarrhea, seizures and shock resulting in death.
What foods are rich in molybdenum?
You can get recommended amounts of molybdenum by eating a variety of foods, including the following:
- 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.
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 are the symptoms of sulfite oxidase deficiency?
Touati et al. (2000) reported 2 unrelated patients with isolated sulfite oxidase deficiency, with a mild clinical course and late onset of symptoms. In 1 patient, the disease started at 15 months with an acute crisis of agitation, unexplained crying, and restlessness following otitis.
Is there a deficiency of sulfite in urine?
In an infant with fatal neurologic disease and ectopia lentis, Mudd et al. (1967) found increased sulfite in the urine with markedly decreased inorganic sulfate excretion. A deficiency in the activity of sulfite oxidase, an enzyme that normally catalyzes conversion of sulfite to sulfate, was postulated.
How is sulfite oxidase involved in the oxidation process?
Specifically, sulfite oxidase is involved in the final step of this process, in which sulfur-containing molecules called sulfites are converted to other molecules called sulfates by adding an oxygen atom (a process called oxidation).