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Werner syndrome

Nowadays those involved in aging research view aging in terms of a genetic disease rather than as a natural, evolution-driven process by which the old make way for the young. A condition of aged friends and relatives seems terrible to conceive; they are afflicted with a ghastly wasting disease, a plague whose effects are inescapable because of our own genes. People plagued with Werner syndrome do not even have the opportunity to experience this natural progression we call aging, instead this disease causes its victims to die of old age by their mid 40's or 50's.

Just about everyone dreads the physical decline often associated with aging, but people with the rare inherited disease known as Werner syndrome have to face the aging process far sooner than most. Unexpectedly, while they are still in their early twenties, their hair grays, their skin loses its suppleness, and their vision clouds from cataracts. Even worse, they get cancer, heart disease, and a host of other diseases that usually don't strike until later in life. Most people afflicted with Werner syndrome die before age 50. Aging experts have long wondered just what kind of molecular defect could cause such a striking acceleration of the


The gene may also yield insights into cancer, because Werner syndrome patients suffer from many forms of cancer or rare tumors. From this, it can be concluded that the Werner syndrome gene is not only an aging gene, but also a cancer gene.

Still, as Schellenberg cautions, answering these questions will not explain everything about aging. "I don't want to sell Werner syndrome as a total mimic of aging," he emphasizes. He also notes that there are some essential differences between how people with Werner syndrome age and the way everyone else ages and not just in the kinds of cancer, they suffer from. Although prematurely old, the patients do not develop Alzheimer's disease or high blood pressure, diseases, which are typically associated with old age. Instead, they experience symptoms not associated with normal aging such as ulceration of the skin, particularly around the ankles, alteration of the vocal chords resulting in a high-pitched voice, and an absence of the growth spurt that normally occurs after puberty.

Adoue, Daniel. Werner's Syndrome. New England Journal of Medicine vol 337. 1997.



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Approximate Word count = 3056
Approximate Pages = 12 (250 words per page double spaced)


  

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