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Chinese medicinal compound stops formation of cysts in polycystic kidney disease in lab

the cells lining the fully-formed kidney tubules keep right on subdividing and growing. The result of this hyperproliferative, unregulated growth: uncontrolled growth of cells lining the tubules and the formation of large cysts in the kidneys.

The vast majority - 85 percent - of patients with polycystic kidney disease have a gene causing PKD1 to be missing or to function poorly. Because most patients inherit only one abnormal gene and one functional gene, the body is usually able to compensate for the faulty gene and the person retains kidney function during the 20s and 30s. But through random mutagenesis, the remaining good copy of PKD1 is lost in some cells, which then switch to the hyperproliferative state. As the person's kidney begins to develop these cysts, the kidney begins to swell, and the person moves to either dialysis or transplant in order to survive.

That means a treatment that slows down the development of cysts does not have to stop their production completely to be effective, says Dr. Crews.

"If we were able to slow the rate of cyst formation by even 10 percent a year, compounded annually, patients would not die from this disease. A relatively small effect would have an enormous clinical benefit," says Dr. Crews.


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Contact: Sylvia Wrobel
ebpress@bellsouth.net
770-270-0989
Federation of American Societies for Experimental Biology
29-Apr-2007


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