Progeria is a rare genetic disorder - only 65 cases in the world - where a child's aging process is accelerated and he or she dies of aging related disease like stroke and heart attack. Life expectancy is about 13 years. While Progeria cannot really be treated but it is helping the health researchers to understand common risks of aging, such as heart attacks and strokes, which account for more than one-third of all U.S. deaths.
Dr. Leslie Gordon, medical director of The Progeria Research Foundation, in an interview to CNN, said that the old age symptoms shown by children are helping them understand how factors like diet and exercise can really impact the aging process, and to what extent diseases like heart attack or heart disease can be impacted by this.
A study published in the Journal of Arteriosclerosis, Thrombosis, and Vascular Biology showed that a protein called progerin increases in all bodies as we grow older, and children who died due to Progeria had very high levels of progerin in their arteries. Their levels compared to that of very old people.
This is prompting research agencies like Buck Institute for Age Research to look into Progeria and Werner syndrome (a premature aging disease that begins in teenage years or early adulthood) to understand the process of normal aging mechanisms. They are focusing on how children with Progeria may not look old but their organs show the same levels of degeneration as that of an old person. Most have very bad cardiovascular condition.
It is only as recent as 2003 that a team of doctors at the National Human Genome Research Institute found a gene mutation responsible for progeria. This gene, LMNA, produces a defective form of a protein called Lamin A , which made the structure of the nucleus unstable, leading to premature aging. It was only after 2003 that a diagnostic test for progeria was developed.