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Marseille/Bethesda (pte020/17.04.2003/12:19) - Through two studies researchers in France and the United States have discovered the reason for Hutchinson-Gilford progeria syndrome (HGPS). The study, which was published in Nature http://www.nature.com and Science http://www.sciencemag.org , found that a single letter change in the genetic code is responsible for premature ageing. Researchers hope that the discovery will speed up the process of test development and therapies.
Hutchinson-Gilford progeria syndrome was first discovered in 1886. Since then 100 cases worldwide have been reported. Symptoms include a remarkable ageing process. Children who suffer from HGPS age 10 times faster than normal children; develop wrinkled skin, lose their hair and are afflicted by sicknesses more common with elderly people, such as joint problems and dislocations. Many die from heart attacks, strokes or cancer before they reach twenty.
Geneticist Francis Collins, director of the National Human Genome Research Institute in Bethesda, Maryland, and leader of the first study, says that it is "the first piece in solving the tragic puzzle of progeria."
Nicolas Levy, of Timone Hospital in Marseille, France, who co-conducted the second study, explains that a correct diagnosis of the disorder will enable doctors to prescribe the appropriate drugs to sufferers. "Identifying the gene responsible for the disease is the first step towards possible therapies," says Levy, though he admits that their development is likely to take some time.
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