To Your Health

The lowdown on amyloidosis

Q. My father and my aunt died from "primary" amyloidosis, and two brothers have the gene, but a third brother does not. What nutrients are lacking that allow it to develop, and what role does heredity play?

A. Amyloidosis is the name given to a group of diseases in which body organs accumulate insoluble proteins called "amyloid" in high enough amounts to impair the functioning of that organ. It is not cancer, but it is a very serious and life-threatening condition. Unfortunately expression of the gene cannot be prevented, and there is no cure for amyloidosis, but you can manage the symptoms and treat the complications.

Amyloidosis is not just one disease. It is a group of diseases, each the result of a mutation in a specific gene affecting a specific organ. The mutation apparently results in formation of misshapen proteins that are not only useless but hard to dispose of because they are insoluble. Imagine a factory producing a product made of nonbiodegradable plastic. A small percentage of these plastic products are sure to be defective, but there is no easy way to do away with them. This is no problem if there are only a few of them. But if a flaw develops in the machinery and a lot of defective plastic products are made with no place to store them, the defective products will accumulate and interfere with the production of all other products. The situation is similar with amyloidosis; the solution is to either slow the production of defective protein or increase the disposal process.

At present there is more optimism that we can enhance the disposal process, since slowing production will probably have to await advances in gene therapy. Destruction of amyloid by very high doses of vitamin C in combination with copper seems to work in animal experiments and may at least prevent amyloidosis from worsening. Low levels of glutathione, another major antioxidant, may also be associated with amyloidosis. Although human studies have not been conducted to evaluate glutathione supplementation for amyloidosis, some clinicians recommend 500 mg two or three times a day to try to prevent the disease.

Bromelain, an enzyme derived from pineapple, may help break down amyloid deposits in kidney tissue; this is suggested by a laboratory study performed on tissue samples from the kidney of one person with a family history of amyloidosis. This preliminary finding does not indicate how this information will translate to treatment or prevention of amyloidosis for people in general.

Even without gene therapy, we may be able to do something about the formation of amyloid. One gene, "apoE4," is known to markedly increase the production of amyloid. Although only a "test tube" experiment, production of insoluble amyloid was reduced in the presence of melatonin, a brain hormone that diminishes with age but that can be easily and safely supplemented.

Although amyloid is an abnormal protein, the amount of protein you eat plays no known role in the development of the disease. Dietary choices and supplements that increase your intake of omega-3 fatty acids theoretically would help to prevent amyloidosis.

Amyloidosis is so uncommon that traveling to a facility with experience in treating it is well worthwhile. The institutions in the U.S. that are most expert in this condition are Boston University, Memorial Sloan-Kettering in New York City, and the Mayo Clinic in Rochester, Minn.

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KEYWORDS FOR THIS STORY

amyloidosis, amyloid

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