Aging: An Unexpected Result from Studying a Rare Syndrome

Note: Aging is usually associated with longevity as well as a decline in physical and mental well-being. Since we all want to “age well,” this health flash, which comes to me personally from my brother Paul, may be of special interest to those of you reading my postings, no matter what your age.

 

Aging & the Progeria Research Foundation (PRF)

My brother Paul is a Professor Emeritus of Microbiology & Immunology at Brown University, Providence, RI.  A former graduate student of his, Leslie Gordon, MD/PhD, conducted her PhD research in the neuro-immunology lab that Paul co-directed at Brown. (Topic: immune responses in the normal brain.)

After graduation, Leslie and her husband, Scott Berns, MD, both with major interests in Pediatric Medicine, received the sad and ironic news that their first baby, Sam, was diagnosed with Progeria at around 6 months of age, a premature aging disease in which all children diagnosed with Hutchinson-Gilford Progeria Syndrome (HGPS) die of “old age symptoms” before age 15, caused by acceleration of the aging process (e.g. heart disease, stroke, arthritis, etc).

Leslie and her family founded The Progeria Research Foundation (PRF) in 1999. It has raised money to support research in this extremely rare disease (one HGPS birth per 4-8 million new births). PRF is managed by family members and others, including her sister Audrey and husband Scott. Research supported by PRF and NIH (National Institutes of Health) has led to the discovery that HGPS is caused by an alteration in a single gene, resulting in the interference with normal maturation of the protein encoded by the genetic DNA. This altered protein was named “progerin” by Leslie.

Dr. Francis Collins, Director of NIH, “often cited the Progeria story of progress from bench to bedside as an example of how a rare disease can be propelled towards cure when there is a non-profit group like PRF effectively collaborating with N.I.H. and others.” (Quoted from the 2010 Annual Report of the Progeria Research Foundation.)

There are approximately 80 known cases of Progeria in 31 countries currently identified by PRF, although experts believe that there are actually 200-250 children worldwide who actually have Progeria. Since over half of them have not yet been identified or diagnosed, PRF has established a global campaign to find the other children. (Source: PRF brochure)

“Studies now confirm the link between Progeria, heart disease and the aging process. Further research on the causes of Progeria and how it can be treated and cured may have benefits for millions of adults who suffer from atherosclerotic heart disease, as well as the entire aging population.” (Direct quote from PRF brochure.)

To learn more about this cutting edge research and its impact on aging, go to www.progeriaresearch.org.

***There is another website for the global campaign to find the remaining 150 unidentified or undiagnosed children with Progeria. That website is www.findtheother150.org.

P.S. Thanks to my brother Paul for all his help in gathering information for this health flash. Also, thanks to the PRG for their excellent literature.


Related Article: At the end of 2008, I reviewed a book by Suza Francina called The New Yoga for Healthy Aging. Click on this link to read the review, but scroll down to the second part of the article, since the first review is of the Lunar Calendar.  (https://www.menupause.info/archives/889).  Here is the cover:

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