Unlocked mysteries of premature-aging syndrome may provide clues to aging

Some exciting news for a few precious little tikes around the world that deserve it very much and so much more. It might also be exciting for the rest of us as well!

Mysteries of early-aging syndrome unlocked, researchers say

Dec. 13, 2005

Courtesy American Society for Cell Biology and World Science staff

Scientists say they are unraveling a longstanding mystery of how a rare syndrome causes its victims to die in their early teens, apparently of old age.

The answer could do more than help those children, researchers say. It could also lead to a better understanding of how normal aging happens, and what if anything one could do to stop it.

An estimated one in 8 million children are born with the condition, called Hutchinson-Gilford Progeria Syndrome. They start life in apparent good health but by six to 18 months develop signs of premature aging, including hair loss, stiff joints, osteoporosis and atherosclerosis. Typically, they die by 13, finished by heart attacks or strokes.

No effective treatments are known, although scientists reported last September that a drug currently being tested against cancer might help the patients.

The cause of the condition, too, remains unknown. But researchers reported one breakthrough in 2003. They traced the condition to a spontaneous mutation in a gene encoding a component of the cell nucleus, the compartment of a cell that stores our genes.

Megan Nighbor, 5, a progeria patient. Her family, which has been appointed as the U.S. Progeria Research Foundation's Ambassador Family for progeria outreach, describes her as a bundle of energy who loves horses. (Photo courtesy of the Markesan Regional Reporter and the Progeria Research Foundation)

See the rest of the story continued here.

Progeria is a despicable disease. I would have to say that in fact it exceeds normal aging in loathsomeness. It robs beautiful children of their lives and time to enjoy life as much as they deserve. Their inner beauty always out-shining their outer, if you've ever had occasion to become familiar with them -- perhaps through a Discovery Channel documentary, they sparkle with joy and hope and love of life, what little of it this terrible disease affords them. We could all take lessons from them on how to appreciate the magical thing that mere existence is.

Let's hope this disease is soon to meet the dustbin of history and along with it that other thief of life that unfortunately plagues everyone plain-old every day aging itself. For even for these precious children, if they were to be cured of their premature aging, that old Dragon-Tyrant awaits them still.