Featured Research

from universities, journals, and other organizations

Adult Stem Cell Changes Underlie Rare Genetic Disease Associated With Accelerated Aging

Date:
March 3, 2008
Source:
NIH/National Cancer Institute
Summary:
Adult stem cells may provide an explanation for the cause of a Hutchinson-Gilford Progeria Syndrome, a rare disease that causes premature aging in children. These findings, the first to indicate a biological basis for the clinical features of HGPS, also known as progeria, may also provide new insights into the biological mechanisms of normal aging.

Adult stem cells may provide an explanation for the cause of a Hutchinson-Gilford Progeria Syndrome (HGPS), a rare disease that causes premature aging in children, according to researchers at the National Cancer Institute (NCI), part of the National Institutes of Health (NIH). These findings, the first to indicate a biological basis for the clinical features of HGPS, also known as progeria, may also provide new insights into the biological mechanisms of normal aging.

Related Articles


"Studies like this of the biology of HGPS hold the potential to benefit children suffering this terrible illness and enlighten us as to the medical changes we all experience as we grow older." said NCI Director John E. Niederhuber, M.D. "As our population ages, we have an increasing need for greater understanding of the biology of aging and age-related illness, such as cancer."

HGPS is an extremely rare hereditary genetic disease of children characterized by signs of premature aging. Children with HGPS generally experience the first symptoms by the age of one, and on average succumb around the age of 15, almost exclusively from premature, progressive heart disease. HGPS occurs in one out of four to eight million births; only 100 patients have been documented in the medical literature. Because its striking cardiovascular effects and other clinical features are so closely associated with the normal aging process, HGPS holds great interest for researchers studying age-related biological changes and disease.

The cause of HGPS, a mutated protein called progerin, was identified in 2003. However, the mechanism by which progerin causes the widespread clinical effects of HGPS has been unclear. To forge this link between molecular biology and medical outcome, Tom Misteli, Ph.D., head of the Cell Biology of Genomes Group at NCI's Center for Cancer Research (CCR), and CCR staff scientist Paola Scaffidi, Ph.D., examined the effects of progerin on gene expression in a laboratory model of HGPS. They found that progerin activates genes involved in the Notch signaling pathway, a major regulator of stem cell differentiation -- the process by which stem cells give rise to the mature cells that make up different tissues.

Because most of the tissues affected by HGPS (e.g., skin, fat, muscles, bone, and blood vessels) arise from a common developmental pathway, Misteli and Scaffidi looked at the effects of progerin on adult mesenchymal stem cells, the common cellular ancestor of these tissue types. An adult stem can renew itself, and can differentiate to yield the major specialized cell types of the tissue or organ. Their experiments revealed that progerin profoundly affects the fate of these stem cells, greatly skewing the rate at which they mature into different tissues.

For instance, progerin-producing stem cells showed accelerated maturation into bone but failed to develop into fat. This could explain two of the distinguishing clinical features of HGPS: abnormal bone growth and an almost complete loss of the fatty tissues normally found just beneath the skin. The researchers were able to mimic the progerin's effects in these stem cells by experimentally activating the same components of the Notch pathway targeted by progerin.

Taken together, the results of these experiments provide a new window into the biology behind the clinical features of HGPS. They may also hold relevance for understanding the biology of normal aging. "Progerin is present at low levels in the cells of healthy people," said Misteli. "One could envision a scenario in which progerin's effects on the Notch pathway and, by extension, on adult stem cells could, over time, lead to many of the tissue changes we commonly associate with the aging process."

Journal reference: Scaffidi P and Misteli T. Lamin A-dependent misregulation of adult stem cells associated with accelerated aging. Nature Cell Biology, March 2008.


Story Source:

The above story is based on materials provided by NIH/National Cancer Institute. Note: Materials may be edited for content and length.


Cite This Page:

NIH/National Cancer Institute. "Adult Stem Cell Changes Underlie Rare Genetic Disease Associated With Accelerated Aging." ScienceDaily. ScienceDaily, 3 March 2008. <www.sciencedaily.com/releases/2008/03/080302150715.htm>.
NIH/National Cancer Institute. (2008, March 3). Adult Stem Cell Changes Underlie Rare Genetic Disease Associated With Accelerated Aging. ScienceDaily. Retrieved March 30, 2015 from www.sciencedaily.com/releases/2008/03/080302150715.htm
NIH/National Cancer Institute. "Adult Stem Cell Changes Underlie Rare Genetic Disease Associated With Accelerated Aging." ScienceDaily. www.sciencedaily.com/releases/2008/03/080302150715.htm (accessed March 30, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Monday, March 30, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

S. Leone in New Anti-Ebola Lockdown

S. Leone in New Anti-Ebola Lockdown

AFP (Mar. 28, 2015) Sierra Leone imposed a three-day nationwide lockdown Friday for the second time in six months in a bid to prevent a resurgence of the deadly Ebola virus. Duration: 01:17 Video provided by AFP
Powered by NewsLook.com
These Popular Antibiotics Can Cause Permanent Nerve Damage

These Popular Antibiotics Can Cause Permanent Nerve Damage

Newsy (Mar. 27, 2015) A popular class of antibiotic can leave patients in severe pain and even result in permanent nerve damage. Video provided by Newsy
Powered by NewsLook.com
WH Plan to Fight Antibiotic-Resistant Germs

WH Plan to Fight Antibiotic-Resistant Germs

AP (Mar. 27, 2015) The White House on Friday announced a five-year plan to fight the threat posed by antibiotic-resistant bacteria amid fears that once-treatable germs could become deadly. (March 27) Video provided by AP
Powered by NewsLook.com
House Ready to Pass Medicare Doc Bill

House Ready to Pass Medicare Doc Bill

AP (Mar. 26, 2015) In rare bipartisan harmony, congressional leaders pushed a $214 billion bill permanently blocking physician Medicare cuts toward House passage Thursday, moving lawmakers closer to resolving a problem that has plagued them for years. (March 26) Video provided by AP
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:

Breaking News:

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile: iPhone Android Web
Follow: Facebook Twitter Google+
Subscribe: RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins