Featured Research

from universities, journals, and other organizations

Uncovering the trail behind growing too old, too soon

Date:
January 25, 2011
Source:
Agency for Science, Technology and Research (A*STAR), Singapore
Summary:
Scientists have produced the world's first human cell model of progeria, a disease resulting in severe premature aging 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.

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. Children with progeria suffer symptoms of premature ageing, including growth retardation, baldness, and atherosclerosis (hardened arteries), and all die in their early teens from either heart attack or stroke.

Scientists from A*STAR's Institute of Medical Biology (IMB) in Singapore and the University of Hong Kong's Department of Medicine 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. Their findings were published this month in the scientific journal, Cell Stem Cell.

Led by IMB's Profs Alan Colman and Colin Stewart, 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. Previously, only mouse models of the disease were available.

Said Prof Colman, "While mouse models of progeria have been informative, no one mouse model recapitulates all the symptoms seen in humans. Our human progeria model allows us to examine the pathology of the disease at a much closer resolution than previously possible."

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.

Background

The group's findings are a significant boost to existing research on over 10 diseases associated with lamin gene mutations. Prof Stewart previously led a study in mice at IMB showing that progeria affected the connective tissues, potentially via defects in a signaling pathway connecting the nuclear lamina with the extracellular matrix and which was associated with death of the smooth muscle in major blood vessels.

Said Prof Stewart, "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."


Story Source:

The above story is based on materials provided by Agency for Science, Technology and Research (A*STAR), Singapore. Note: Materials may be edited for content and length.


Journal Reference:

  1. Jinqiu Zhang, Qizhou Lian, Guili Zhu, Fan Zhou, Lin Sui, Cindy Tan, Rafidah Abdul Mutalif, Raju Navasankari, Yuelin Zhang, Hung-Fat Tse. A Human iPSC Model of Hutchinson Gilford Progeria Reveals Vascular Smooth Muscle and Mesenchymal Stem Cell Defects. Cell Stem Cell, 2011; 8 (1): 31 DOI: 10.1016/j.stem.2010.12.002

Cite This Page:

Agency for Science, Technology and Research (A*STAR), Singapore. "Uncovering the trail behind growing too old, too soon." ScienceDaily. ScienceDaily, 25 January 2011. <www.sciencedaily.com/releases/2011/01/110124102948.htm>.
Agency for Science, Technology and Research (A*STAR), Singapore. (2011, January 25). Uncovering the trail behind growing too old, too soon. ScienceDaily. Retrieved September 17, 2014 from www.sciencedaily.com/releases/2011/01/110124102948.htm
Agency for Science, Technology and Research (A*STAR), Singapore. "Uncovering the trail behind growing too old, too soon." ScienceDaily. www.sciencedaily.com/releases/2011/01/110124102948.htm (accessed September 17, 2014).

Share This



More Health & Medicine News

Wednesday, September 17, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

President To Send 3,000 Military Personnel To Fight Ebola

President To Send 3,000 Military Personnel To Fight Ebola

Newsy (Sep. 16, 2014) President Obama is expected to send 3,000 troops to West Africa as part of the effort to contain Ebola's spread. Video provided by Newsy
Powered by NewsLook.com
Man Floats for 31 Hours in Gulf Waters

Man Floats for 31 Hours in Gulf Waters

AP (Sep. 16, 2014) A Texas man is lucky to be alive after he and three others floated for more than a day in the Gulf of Mexico when their boat sank during a fishing trip. (Sept. 16) Video provided by AP
Powered by NewsLook.com
Ivorians Abandon Monkey Pets in Fear Over Ebola Virus

Ivorians Abandon Monkey Pets in Fear Over Ebola Virus

AFP (Sep. 16, 2014) Since the arrival of Ebola in Ivory Coast, Ivorians have been abandoning their pets, particularly monkeys, in the fear that they may transmit the virus. Duration: 00:47 Video provided by AFP
Powered by NewsLook.com
Study Links Male-Pattern Baldness To Prostate Cancer

Study Links Male-Pattern Baldness To Prostate Cancer

Newsy (Sep. 16, 2014) New findings suggest men with a certain type of baldness at age 45 are 39 percent more likely to develop aggressive prostate cancer. Video provided by Newsy
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:
from the past week

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