Rare Aging Disease in Children, Progeria, Linked to
Aging in General Population
There may be a tie between the deadly heart disease
of Progeria and the heart disease that affects us all as we age
Sept. 7, 2010 The rare fatal genetic heart
disease characterized by the heart-breaking appearance of accelerated
aging in children, and causing an average lifespan of just 13 years, is
known as Progeria. A new study finds that progerin, the abnormal protein
that causes Progeria, is also present in people without the disease and
it increases with age.
Progeria, also known as Hutchinson-Gilford Progeria
Syndrome (HGPS), causes all children with the disease to die of the same
heart disease that affects millions of normal aging adults
(atherosclerosis), but instead of occurring at 60 or 70 years of age,
these children may suffer heart attacks and strokes even before age 10,
and the average age of death is 13 years.
Post-meal blood vessel expansion occurs in young,
not old; Muscles of young people look 50 years older by making muscle
blood vessels behave as they do in seniors
The scientist involved in this news study see a
possibility that finding treatments and a cure for Progeria may provide
clues to preventing or treating heart disease in the general population,
too.
On August 26, 2010, Arteriosclerosis,
Thrombosis, and Vascular Biology electronically published, ahead of
print, the results of the study comparing Progeria, and typical
cardiovascular aging, entitled "Cardiovascular Pathology in
Hutchinson-Gilford Progeria: Correlation With the Vascular Pathology of
Aging".
This study discovered that progerin is present in
the vasculature of the general population and increases as people grow
older.
Researchers examined cardiovascular autopsies and
progerin distribution in two patients with Progeria along with 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 the 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
is a contributor 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
More about Progeria
The Progeria Research Foundation (PRF) was
established in 1999 to find the cause, treatment and cure for Progeria
a rapid aging disease that causes children to die from heart disease or
stroke at an average age of 13. In the past 10 years, research conducted
in partnership with PRF has identified the gene that causes Progeria and
possible treatments. PRF is now funding first-ever Progeria clinical
drug trials, currently underway at Children's Hospital Boston. To learn
more about Progeria and what you can do to help, please visit
progeriaresearch.org
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