Hutchinson Gilford Progeria Syndrome

Imagine growing old before having the chance to grow up. Hutchinson-Gilford Syndrome, commonly referred to as progeria, is a disease that has this effect on a significant amount of children around the world. Although there are many different forms of progeria, Hutchinson-Gilford Progeria Syndrome is the classic type. The name progeria comes from Greek origin and means “prematurely old”. Those who suffer from this disease take on the effects of early aging. Although their bodies begin to age early in life, children with progeria maintain an intelligence level that is typical of a child who does not have the disease. Scientists have not been able to develop a cure for this disease and therefore children with progeria only live to about age thirteen. Due to a genetic mutation detected through clinical examination and blood testing, a small percentage of young children suffer from progeria, a rare yet nonhereditary, fatal condition that causes
This gene produces a lamin A protein which is responsible for holding a cell’s nucleus together and keeping it stable. Researchers found that progerin is the abnormal lamin A protein that causes progeria. It is also linked to telomere dysfunction, or cellular aging, which causes the nucleus in a cell to turn unstable ("Progeria 101/FAQ"). Researchers believe that this defect leads to the aging process of those who suffer from progeria. Scientists have now discovered the relationship between the general aging population and children with progeria. Each person makes a small amount of progerin. Although every body produces this protein, children with progeria make much more than those who are not affected by this disease. In the average person, the protein builds up over time and is seen to be partially responsible for the symptoms of aging ("Progeria 101/FAQ"). Since people with progeria have a large amount of this protein from a young age, they are predisposed to early