Progress in Progeria Research

The accelerated aging condition progeria so far appears to be an extreme example of one type of cellular damage that usually only provides a very modest contribution to degenerative aging. Here is news of recent research: "Hutchinson-Gilford Progeria Syndrome, also known as progeria, is caused by a mutation in the gene encoding for the protein lamin A, an important component of the membrane surrounding a cell's nucleus. The mutation results in a truncated form of lamin A called progerin, which in turn causes misshapen cell nuclei and DNA damage. ... [researchers] have produced the world's first human cell model of progeria, a disease resulting in severe premature ageing in one in four to eight million children worldwide. This model has allowed them to make new discoveries concerning the mechanism by which progeria works. ... the team used a novel technique of deriving induced pluripotent stem (iPS) cells from cells of human progeria patients. This human progeria model allows the group to trace and analyse the distinctive characteristics of progeria as it progresses in human cells. ... The researchers used their iPS cells to identify two types of cells - mesenchymal stem cells (MSCs) and vascular smooth muscle cells (VSMCs) - that were particularly adversely affected by progeria. This means that a young patient with progeria would typically have fewer MSCs and VSMCs than other children. MSCs were found to be very sensitive to a low oxygen environment and their losses could delay renewal of the various tissues they gave rise to, thus exacerbating the patient's symptoms of ageing. The same effect on VSMCs could explain why their number was reduced in the patient's heart vessels. ... This new study provides further evidence for the role of lamin processing in connective tissue function, as well as insights into the normal ageing process. We hope to soon find new routes of intervention to treat this incurable disease. Such interventions may be of use in treating atherosclerosis in general, a condition afflicting many millions of individuals."

Link: http://www.sciencedaily.com/releases/2011/01/110124102948.htm

Comments

Okay My Name Is Talia Nicole Castillo.!
I Am Only 14,i've been doing research of progeria for personal reason's.!!
my reason's are all out of concern and worrying. i wish i could save one life i would give mine up to at least let one progeria patient survive and live the life they deserve.! i am trying to go around school and get a project done to support this cause like cancer a cure must be found.!! i have srong feeling to change the world i want to be that young child who succeeds in the process of their dream to help anyone in need.! even meeting a progeria patient would let me come to a better understanding seeing things in another perspective. my life would change i would literally be honored.!

Posted by: talia castillo at February 6th, 2012 10:24 AM

Talia:
I understand, I feel the same way, too.
Helping out can be really simple. A good way to start is helping to fundraise.
In my own research, I found ProgeriaResearch.org. They have a program where you can order cans for people to put change in, like at supermarkets and convenience stores at the checkout. If you make sure to ask your guardian first, it's an easy way to get involved and help out. :) And, if you feel REALLY strongly about it, why not ask for donations to a research facility for your next birthday? I did that once for the Leukemia and Lymphoma society, and it was totally worth not getting any presents.

Here's the link to the coin drive. Make sure to get your guardian check it out and see if it's worth it.
http://www.progeriaresearch.org/coins_to_cure.html

Good luck!

Melanie

Posted by: Melanie at February 17th, 2012 12:29 PM

Hi. I am fifteen years old. I am doing a research report on Progeria. I have read many explanations of the scientific research aspect on countless websites. Unfortunately, as I am in tenth grade and do not have quite the right scientific background yet, a lot of their scientific jargon flew pretty much in one ear and out the other. I find this topic quite fascinating and would love to have the new developments explained to me in a clear and concise way.
Can you assist me?

Posted by: F. Dawn at May 7th, 2012 5:18 PM

i am doing research on progeria... its really interesting. i feel bad for the kids, but there will be a permanent cure eventually based on all that i have researched about the disorder. i need more info about it so please email me with more info. thanks.

Posted by: Amanda Morgan at October 8th, 2014 11:12 AM

HII
I AM AN 23 YRS OLD GIRL AND IAM DOING MY BACHELOR STUDIES IN PHARMACY
AND I HAVE CHOOSED PROGERIA AS MY RESEARCH TOPIC , UNFORTUNATELY AT THE BEGINNING I WAS NOT AWARE ABOUT THE CONDITIONS ANS SERIOUSNESS BUT NOW I REALLY WANNA DO BESTEST FOR THEM AND ALSO, IN INDIA THE PEOPLES ARE LESS COMMON WITH THIS TERM , MY PRESENTATION IS YET TO COME ON DECEMBER 2016 , SO IT WILL BE THE GOLDEN CHANCE FOR ME TO AWARE THE PEOPLE, ESPECIALLY THE STUDENTS ABOUT PROGERIA, SO I REQUEST YOU KIND HELP ON DESCRIBE ABOUT LATEST CLINICAL TRIALS AND FUTURE PLANS IN RESEARCH AND TREATMENT OF PROGERIA.
THANKING TOU
MAJIDA

Posted by: majida at November 29th, 2016 3:44 AM

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