Progeria Linked to Progerin and Aging

Hutchinson-Gilford Progeria Syndrome (Progeria)

Hutchinson-Gilford Progeria Syndrome, also known as Progeria, is a specific type of progeroid syndrome. Progeroid syndromes are a group of diseases that causes individuals to age faster than usual, making them appear older. Children with progeria usually develop the first symptoms during their first few months. Affected children typically look normal at birth and early infancy but grow more slowly than other children and do not gain weight at the expected rate (failure to thrive). They develop a characteristic facial appearance, including prominent eyes, a thin nose with a beaked tip, thin lips, a small chin, and protruding ears. The earliest symptoms may include a failure to thrive and a localized scleroderma-like skin condition. Patients born with progeria typically live to mid-teens or early twenties.

Main Digest

The rare aging disease, Progeria, linked to aging in the general population - Study results demonstrate progerin increases in blood vessels as we age.

Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS), is a rare, fatal genetic disease characterized by an appearance of accelerated aging in children. All children with Progeria die of the same heart disease that affects millions of normal aging adults (atherosclerosis). Still, instead of occurring at 60 or 70, these children may suffer heart attacks and strokes even before age 10, and the average age of death is 13 years. Finding treatments and a cure for Progeria may provide clues to preventing or treating heart disease in the general population.

On August 26, 2010, Arteriosclerosis, Thrombosis, and Vascular Biology electronically published, ahead of print, the results of a study comparing Progeria, and typical cardiovascular aging, entitled "Cardiovascular Pathology in Hutchinson-Gilford Progeria: Correlation With the Vascular Pathology of Aging". The study found that progerin, the abnormal protein that causes Progeria, is also present in the vasculature of the general population and increases with age.

Researchers examined cardiovascular autopsies and progerin distribution in two patients with Progeria and a group of 29 individuals without Progeria. Between the ages of one month and 97 years, researchers found that progerin in individuals without Progeria increased an average of 3.3 percent per year in the coronary arteries.

"We found similarities between many aspects of cardiovascular disease in both Progeria and atherosclerosis that affects millions of people throughout the world," said Dr. Leslie Gordon, senior author of the study and Medical Director of The Progeria Research Foundation. "We also found progerin in cardiac blood vessels of people without Progeria. The fact that progerin is present at all tells us that there may be a tie between the heart disease of Progeria and the heart disease that affects us all as we age."

This study supports the possibility that progerin contributes to the risk of atherosclerosis in the general population and merits examination as a potential new element influencing vascular health with aging.

"By examining one of the rarest diseases in the world, we are gaining crucial insight into a disease that affects millions of people worldwide. Ongoing research has the potential to have a significant impact on our understanding of heart disease and aging," said Dr. Gordon

The Progeria Research Foundation (PRF) was established in 1999 to find the cause, treatment, and cure for Progeria. This rapid aging disease causes children to die from heart disease or stroke at 13. Research in partnership with PRF has identified the gene that causes Progeria and possible treatments in the past ten years. PRF is now funding first-ever Progeria clinical drug trials, currently underway at Children's Hospital Boston.

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This quality-reviewed publication titled "Progeria Linked to Progerin and Aging" was chosen for publishing by Disabled World's editors due to its relevance to our readers in the disability community. While the content may have been edited for style, clarity, or brevity, it was originally authored by Progeria Research Foundation (PRF) and published 2010/09/07 (Edit Update: 2022/06/24). For further details or clarifications, you can contact Progeria Research Foundation (PRF) directly at progeriaresearch.org. Please note that Disabled World does not provide any warranties or endorsements related to this article.

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Cite This Page (APA): Progeria Research Foundation (PRF). (2010, September 7). Progeria Linked to Progerin and Aging. Disabled World. Retrieved April 19, 2024 from www.disabled-world.com/health/aging/progeria-aging.php

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