Researchers also will study the structure and function of the copper-transporting ATPases, which are central to the copper metabolism and, when mutated, are associated with Menkes and Wilson's diseases.

Menkes disease occurs when dietary copper is trapped in intestinal cells and is abnormally low in tissues. According to the National Institute of Neurological Disorders and Stroke, Menkes infants, mostly males, are born prematurely and suffer from stunted development, as well as seizures, failure to thrive, low body temperature, and kinky, colorless and fragile hair. There is no cure for the disease and it is usually fatal by age 10.

Wilson's disease is caused when excessive copper accumulates in the body, leading to liver disease in about 40 percent of patients as well as neurological problems, including tremors, rigidity, drooling, difficulty with speech, abrupt personality change, and unusual behavior associated with neurosis and psychosis. If untreated, it is generally fatal by age 30.

Iron metabolism and its link to hemochromatosis is being studied as well by OHSU investigators. An inherited disease, hemochromatosis occurs when the body absorbs and stores too much iron, allowing it to build up in the liver, heart and pancreas and triggering their failure. Caroline Enns, Ph.D., professor of cell and developmental biology in the OHSU School of Medicine, is tracking the defective gene that causes the disease.

"There is a very tight link between copper and iron metabolism," Lutsenko said. "Studying the systems in parallel is mutually beneficial. The experimental approaches will be very similar and we expect to discover interesting connections."

The metal ion project began earlier this year following grants from the Oregon Opportunity medical research funding effort and the National Institute of General Medical Sciences. It is t
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Contact: Jonathan Modie
modiej@ohsu.edu
503-494-8231
Oregon Health & Science University
13-Jan-2004