Progeria: aging too fast
Photo courtesy of The Progeria Research Foundation, Inc. and the Barnett Family
Both Werner's Syndrome and HGPS are examples of progeria, which means 'prematurely
old.' Progeria isn't exactly rapid aging; people with progeria just look and perhaps
feel like they're quickly growing old.
What's Werner's Syndrome?
Werner's Syndrome is a rare illness that appears to age the sufferer at twice the
normal rate. Those who suffer from Werner's Syndrome show full symptoms in their
twenties, and die usually in their thirties or forties, gray-haired and wizened,
with multiple conditions such as cancer, heart disease, and osteoporosis.
What's Hutchinson-Gilford Progeria Syndrome?
Hutchinson-Gilford Progeria Syndrome, or HGPS, is an even rarer illness that appears
within the first year of life. Children with HGPS have dwarfism, baldness, a pinched
nose and small, narrow face, and dry, scaly skin. They tend to develop stiff joints,
hip dislocations, as well as atherosclerosis (clogged arteries) that causes strokes
or heart problems. They rarely live past their early teens.
Why does it happen?
In the case of Werner's Syndrome, symptoms of aging are caused by a problem inside
the body's cells. Most cells can divide many times before they die, but as a person
grows older, their cells divide fewer times. People with Werner's Syndrome begin
life with cells that divide much less often than normal, so they appear to "age"
quickly.
Werner's Syndrome appears to be caused by a defect in the gene that provides instructions
to a protein called a helicase. Helicases unwind the DNA double-helix that's in
almost every cell of the body, so that it can be copied or repaired. Dividing cells
copy DNA each time they divide, which seems to be why Werner's Syndrome doesn't
affect the brain or muscles—those cells hardly ever divide.
Is there a cure?
Scientists suspect aging is caused by accumulating damage to cells, and especially
to DNA, and Werner's Syndrome supports that theory. But no one knows what gene causes
HGPS, and there's currently no cure for either condition.