Rare Progeria helps comprehend aging

One of the rarest disorders in medical history may now hold the key to our understanding of age-related heart disease in general populations. Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS), is characterized by rapid aging among children who often succumb to the disorder at around 13 years. Most deaths in HGPS cases occur due to heart attacks or strokes. Now scientists from The Progeria Research Foundation have discovered that the rare condition actually has correlations with common vascular disorders in aging populations.

Published in Arteriosclerosis, Thrombosis, and Vascular Biology, August 2010, the study detects the presence of progerin, the protein responsible for Progeria, in the blood vessels of the general population. It also detected that progerin increased with age in general populations.  In a prepared statement, Dr Leslie Gordon, Medical Director of The Progeria Research Foundation said “We found similarities between many aspects of cardiovascular disease in both Progeria and the atherosclerosis that affects millions of people throughout the world. 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.”

However, this study examined data from autopsies of two HGPS cases and 29 individuals without Progeria. The sample size may have proven consistent results but would have to be expanded to further the understanding of this correlation. Dr Gordon adds that “the main thrust of the paper’s findings was detecting  progerin in the general population. We will need to do more research to further explore the possibility of using this correlation to treat general cardiovascular disorders.”

Bibliography:

Michelle Olive ; Ingrid Harten ; Richard Mitchell ; Jeanette Beers ; Karima Djabali ; Kan Cao ; Michael R. Erdos ; Cecilia Blair ; Birgit Funke ; Leslie Smoot ; Marie Gerhard-Herman ; Jason T. Machan ; Robert Kutys ; Renu Virmani ; Francis S. Collins ; Thomas N. Wight ; Elizabeth G. Nabel ; and Leslie B. Gordon, Cardiovascular Pathology in Hutchinson-Gilford Progeria: Correlation With the Vascular Pathology of Aging , Arteriosclerosis, Thrombosis, and Vascular Biology, Aug 2010;doi:10.1161/ATVBAHA.110.209460



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