Copper in the Brain
Trace amounts of metals nourish body and brain, but, in excess, metals can do harm. In Wilson's Disease (WD), copper accumulates to toxic levels in several organs, especially liver and brain. In the brain, the copper deposits cause profound psychiatric and neurological symptoms. WD is fatal unless treated with agents to remove the copper, treatment that is only moderately successful.
Last December, Rudolph Tanzi and colleagues at Massachusetts General Hospital isolated the recessive gene that causes WD. In August, they reported another set of studies finding that both copper and zinc bind tightly to a protein associated with characteristic brain deposits of Alzheimer's disease. That finding sent the investigators searching for genes that could be responsible for metal-binding, a search that led them back to the WD gene. They found that the WD gene encodes a protein responsible for copper transport in cells throughout the body, but occurring at high levels in liver and brain. They also found at least six mutations of the gene that can affect the "domains" regulating copper transport and cause a copper buildup in cells. The WD gene also shows a striking similarity to a protein involved in Menkes' disease, another metabolic disorder involving a shortage of copper in cells that need it. The findings may both improve diagnosis of the two diseases and open the door to developing better treatments than those currently in use.
The Wilson's Disease gene encodes the "domains" that regulate copper transport out of cells; when transport falters, copper can bind to at least five sites (left) in cells.
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