|Molybdenum Cofactor Deficiency||Homocystinuria||Marfan
|Linked to unusual sulfur metabolism?||YES||YES||?|
Molybdenum, sulfur and copper share a complex interrelationship. They each are cofactors for the other two. This is known as the copper-molybdenum-sulfur triangle. Imbalances of one of these nutrients can cause imbalances in the other two. The nutritional aspects of this triangle has been studied extensively in animals, but unfortunately less so in humans. Perhaps the relationship may also play a role in people with dislocated lenses, including those diagnosed with Marfan syndrome.
Interestingly, patients with homocystinuria have been found to have abnormal levels of copper. That means the disorder has biochemical links to both copper and sulfur, two out of the three elements in the copper-molybdenum-sulfur triangle. Do patients with homocystinuria have abnormalities of molybdenum, too? It would be interesting to find out.
Patients with dislocated lenses caused by molybdenum cofactor deficiencies and isolated sulfite oxidase deficiencies usually have low uric acid levels. Interestingly, molybdenum is known to raise uric acid levels, which is why people with gout, a condition of too high uric acid levels, are told to avoid molybdenum supplements.
Foods high in purines such as broth, asparagus, gravy, mushrooms and organ meats also raise uric acid levels and are also bad for gout. It would be interesting to find out what effect molybdenum rich foods and/or a diet high in purines aimed at raising uric acid levels would have on people with dislocated lenses caused by molybdenum cofactor deficiencies and isolated sulfite oxidase deficiencies. It would also be interesting to find out if Marfans have low uric acid levels, too, and if they do, what effect a diet aimed at raising uric acid levels would have on their conditions, especially on Marfan patients with dislocated lenses.
Dislocated lenses have a variety of known causes besides Marfan syndrome. Three of the other known causes of dislocated lenses, molybdenum cofactor deficiencies, isolated sulfite oxidase deficiencies and homocystinuria, are all associated with abnormalities of sulfur amino acid metabolism. Besides dislocated lenses, all three of these disorders plus Marfan syndrome all share the feature of the Marfan habitus - tall, slender with long limbs and skeletal defects such as pectus deformities and scoliosis.
Could Marfan syndrome be related to a problem with sulfur metabolism, too? Could it be treated by dietary modifications? People have told me it is not possible for Marfan syndrome to be treatable through diet because is a genetic disorder. I disagree that that is a valid conclusion because many genetic disorders, including homocystinuria, have been found to be treatable through dietary modifications. Additionally, the case for Marfan syndrome being caused by mutations in fibrillin genes alone is illogical, considering that a large number of studies conducted over the past several years have consistently shown that most Marfans do not even have fibrillin mutations, and that many people who have fibrillin mutations don't have Marfan syndrome. (See my section on fibrillin for more on this subject.)
Perhaps, like sulfite oxidase deficiencies and homocystinuria, Marfan syndrome can be impacted by both diet and genes. It seems unlikely to me that the similarities between the three disorders and molybdenum cofactor deficiencies are all due to random coincidence. It is reasonable to conclude that they all may be biochemically related. Sulfur metabolism abnormalities are the common link between homocystinuria, molybdenum cofactor deficiency and isolated sulfite oxidase deficiency. Perhaps it is also a common link between those three disorders and Marfan syndrome, too.
Beyond those features that that are covered in this section, many of the other features of Marfan syndrome also have nutritional links. These include calcification of the heart valves and mitral valve prolapse.
Related sections of interest:
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