Featured Research

from universities, journals, and other organizations

New insight into 'accelerated aging' disease

Date:
September 13, 2010
Source:
Cell Press
Summary:
Hutchinson-Gilford Progeria Syndrome (HGPS or progeria) is a rare genetic disease that causes young children to develop symptoms associated with advanced age, such as baldness, wrinkles, osteoporosis and cardiovascular disease. Now, a study uses a mouse model to shed light on progeria, and perhaps also on the normal aging process.

Hutchinson-Gilford Progeria Syndrome (HGPS or progeria) is a rare genetic disease that causes young children to develop symptoms associated with advanced age, such as baldness, wrinkles, osteoporosis and cardiovascular disease. Now, a study published by Cell Press in the September 14th issue of the journal Developmental Cell uses a mouse model to shed light on progeria, and perhaps also on the normal aging process.

Progeria is caused by a mutation in the gene for lamin A that leads to production of "progerin," a truncated form of the lamin A protein that causes the cell nucleus to become misshapen. "How progerin causes progeria and whether it contributes to the normal aging process are areas of intense speculation," says senior study author, Dr. Colin L. Stewart from the Institute of Medical Biology in Singapore.

Dr. Stewart and colleagues had previously developed a mouse model for progeria. In the current study, they showed that the mutation associated with their mouse model produces a progerin-like truncation of lamin A and causes post-natal connective tissue cells to stop producing an extracellular matrix. The lack of this surrounding matrix then causes the cells to stop dividing and to die. However, the researchers did not see the same effects when they studied embryonic cells. This difference between pre-natal and post-natal effects on cell behavior in the mutant mice is significant because children with progeria appear normal at birth but develop signs of accelerated aging soon after, often dying from heart disease while they are still in their teens.

The researchers go on to show that the defects in the extracellular matrix in mouse and human progeria cells are due to abnormalities in a protein network called the Wnt signaling pathway. "Our results provide support for the hypothesis that progeria is a disease of the connective tissue extracellular matrix which manifests as abnormalities in the skeleton, teeth, skin and vasculature," concludes Dr. Stewart. "If these failures are due to defective Wnt signaling and/or cytoskeletal-extracellular matrix function, they suggest possible new routes of intervention that may help in treating this disease."

As there is also evidence for defective lamin production in the vascular system during the normal aging process, the researchers are keen to explore potential implications of their new findings in these and other aspects of both progeria and normal aging.

The researchers include Lidia Hernandez, NCI, Frederick, MD, Medical Oncology Branch, Center for Cancer Research, NCI, Bethesda, MD; Kyle J. Roux, University of Florida College of Medicine, Gainesville, FL; Esther Sook Miin Wong, Institute of Medical Biology, Immunos, Singapore; Leslie C. Mounkes, NCI, Frederick, MD; Rafidah Mutalif, Institute of Medical Biology, Immunos, Singapore; Raju Navasankari, University of Florida College of Medicine, Gainesville, FL, Institute of Medical Biology, Immunos, Singapore; Bina Rai, Institute of Medical Biology, Immunos, Singapore; Simon Cool, Institute of Medical Biology, Immunos, Singapore; Jae-Wook Jeong, Baylor College of Medicine, Houston, TX; Honghe Wang, NCI, Frederick, MD; Hyun-Shik Lee, NCI, Frederick, MD; Serguei Kozlov, NCI, Frederick, MD; Martin Grunert, Institute of Medical Biology, Immunos, Singapore; Thomas Keeble, Institute of Medical Biology, Immunos, Singapore; C. Michael Jones, Institute of Medical Biology, Immunos, Singapore; Margarita D. Meta, University of California, Los Angeles, CA; Stephen G. Young, University of California, Los Angeles, CA; Ira O. Daar, NCI, Frederick, MD; Brian Burke, University of Florida College of Medicine, Gainesville, FL; Alan O. Perantoni, NCI, Frederick, MD; and Colin L. Stewart, NCI, Frederick, MD, Institute of Medical Biology, Immunos, Singapore.


Story Source:

The above story is based on materials provided by Cell Press. Note: Materials may be edited for content and length.


Journal Reference:

  1. Lidia Hernandez, Kyle J. Roux, Esther Sook Miin Wong, Leslie C. Mounkes, Rafidah Mutalif, Raju Navasankari, Bina Rai, Simon Cool, Jae-Wook Jeong, Honghe Wang, Hyun-Shik Lee, Serguei Kozlov, Martin Grunert, Thomas Keeble, C. Michael Jones, Margarita D. Meta, Stephen G. Young, Ira O. Daar, Brian Burke, Alan O. Perantoni, Colin L. Stewart. Functional Coupling between the Extracellular Matrix and Nuclear Lamina by Wnt Signaling in Progeria. Developmental Cell, 2010; 19 (3): 413-425 DOI: 10.1016/j.devcel.2010.08.013

Cite This Page:

Cell Press. "New insight into 'accelerated aging' disease." ScienceDaily. ScienceDaily, 13 September 2010. <www.sciencedaily.com/releases/2010/09/100913121604.htm>.
Cell Press. (2010, September 13). New insight into 'accelerated aging' disease. ScienceDaily. Retrieved September 23, 2014 from www.sciencedaily.com/releases/2010/09/100913121604.htm
Cell Press. "New insight into 'accelerated aging' disease." ScienceDaily. www.sciencedaily.com/releases/2010/09/100913121604.htm (accessed September 23, 2014).

Share This



More Health & Medicine News

Tuesday, September 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Liberia Pleads for Help to Fight Ebola

Liberia Pleads for Help to Fight Ebola

AP (Sep. 22, 2014) Liberia's finance minister is urging the international community to quickly follow through on pledges of cash to battle Ebola. Bodies are piling up in the capital Monrovia as the nation awaits more help. (Sept. 22) Video provided by AP
Powered by NewsLook.com
Ebola Doctor Says Border Controls Critical

Ebola Doctor Says Border Controls Critical

AP (Sep. 22, 2014) A Florida doctor who helped fight the expanding Ebola outbreak in West Africa says the disease can be stopped, but only if nations quickly step up their response and make border control a priority. (Sept. 22) Video provided by AP
Powered by NewsLook.com
Global Ebola Aid Increasing But Critics Say It's Late

Global Ebola Aid Increasing But Critics Say It's Late

Newsy (Sep. 21, 2014) More than 100 tons of medical supplies were sent to West Africa on Saturday, but aid workers say the global response is still sluggish. Video provided by Newsy
Powered by NewsLook.com
Sierra Leone in Lockdown to Control Ebola

Sierra Leone in Lockdown to Control Ebola

AP (Sep. 21, 2014) Sierra Leone residents remained in lockdown on Saturday as part of a massive effort to confine millions of people to their homes in a bid to stem the biggest Ebola outbreak in history. (Sept. 20) 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