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Research Reveals How Down Syndrome Shields Against Cancer
Date:5/20/2009

m, a cancer researcher with the Children's Vascular Biology Program at Children's Hospital, Boston. "That's not true now. Many medical issues [for people with Down syndrome] are treatable and correctable, and they now live long enough. And we still find significant protection against solid-tumor cancers."

This study builds on the work of U.S. angiogenesis pioneer Dr. Judah Folkman, who died last year. Angiogenesis refers to the growth of blood vessels necessary to nourish cancer cells. Folkman hypothesized that angiogenesis inhibition might be key to preventing cancers from forming.

Folkman's initial theory stemmed partly from the observation that people with Down syndrome also suffered fewer angiogenesis-related diseases such as diabetic retinopathy, atherosclerosis and the eye disease macular degeneration.

These researchers zeroed in on the Dscr1 (Down syndrome candidate region-1) gene, which dampens the growth of blood vessels by inhibiting a chemical called vascular endothelial growth factor.

The gene's activity is elevated in mouse models of Down syndrome and in human Down syndrome tissue. This elevation in activity is enough to suppress tumors, although it doesn't explain the extent of the cancer-protective effect seen in individuals with Down's, the researchers said.

"This suggests that Dscr1 is incredibly important in blocking tumor growth, and the reason the tumors grow slowly is that blood vessel formation was blocked," Ryeom said. "This told us that Dscr1 played a critical role in suppressing tumor angiogenesis."

Dscr1 alone resulted in some tumor suppression but there may be as many as five genes involved in the action. "Dscr1 is necessary but not sufficient," Ryeom explained.

In fact, Ryeom and her colleagues believe that Dscr1 works with another gene on chromosome 21, called Dyrk1a, to block what's known as the "calcineurin-signaling pathway" -- a circuit involved in helping tu
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