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Tufts E-News --Beyond Their Years

Tufts E-News --Beyond Their YearsHaving recently isolated the gene which causes progeria, two sisters – one a researcher at Tufts, the other a Tufts graduate – are working to fight the rapid-aging syndrome. Boston.

Boston [05.13.03] When Dr. Leslie Gordon's son Sam was diagnosed with progeria - a rare illness which causes rapid aging in children - four years ago, the researcher in the Anatomy and Cell Biology department of Tufts School of Medicine discovered that virtually no information existed on the disease. So she and her sister Audrey - a Tufts graduate - formed a foundation dedicated to finding a cure for the syndrome. Now, thanks to their work, an international research team lead by Dr. Leslie Gordon recently isolated the gene believed to cause progeria, a giant first step to defeating the disease.

"It's an incredible, incredible moment for us," Gordon told The Boston Globe. "There's hope for Sam. There's hope for all children with progeria."

Progeria is the Greek term for prematurely old. Patients with the disease develop dwarfism, baldness, and heart problems. On average, they live only 13 years.

By collecting and analyzing tissue samples from progeria patients around the world, Gordon's team of researchers traced the disease to a gene called LMNA. If born with a DNA error in that gene, children will age at 10 times the normal rate.

"We have shown we can find the shorter, defective protein causing all these problems and leading to this disease in children," Gordon told Reuters. "So we have a place to start now."

According to Gordon, a structural abnormality is the cause of the problem.

"The nucleus is usually a nice, round structure," Gordon told Reuters. "There are these bubbles that form because of this defect. That most likely causes the instability that leads to cell death."

Gordon, who hopes to develop a test for the disease soon, told CNN that the breakthrough is only the tip of the iceberg of progeria research - which many hope will have broader applications for the problems of aging.

"Isolating the gene is just the beginning," Gordon told CNN. "It is our goal to find treatments and possibly a cure for this rare, life-threatening disease that robs children of their adulthood."

It is also just the start of what Gordon is doing to fight progeria. She has also co-founded the Progeria Research Foundation - an organization dedicated to raising awareness and education the public about the syndrome - with her sister, Audrey. A lawyer and Tufts graduate, Audrey Gordon is President and Executive Director of the foundation.

"Our family pooled its resources and formed this Foundation, the first of its kind," Audrey Gordon wrote on the foundation's website.

The organization recently held a fundraising gala hosted by husband and wife actors Ted Danson and Mary Steenburgen, which raised $175,000 for progeria research.

Leslie Gordon said she optimistic about future of their work.

"I feel like the door has flown open," Leslie Gordon said in a story which appeared in the Washington Post. "It's going to be a springboard for a lot of exciting research for aging and heart disease and, obviously, will be a big step for a cure for these children."

 

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