Featured Research

from universities, journals, and other organizations

Accelerated aging in children: Promising treatment for progeria within reach

Date:
May 16, 2013
Source:
University of Gothenburg
Summary:
Pharmaceuticals that inhibit a specific enzyme may be useful in treating progeria, or accelerated aging in children. A new study indicates that the development of progeria in mice was inhibited upon reducing the production of this enzyme.

This is professor Martin Bergö, Sahlgrenska Cancer Center, University of Gothenburg
Credit: Sahlgrenska Academy

Pharmaceuticals that inhibit a specific enzyme may be useful in treating progeria, or accelerated aging in children. As reported in the journal Science, a new study performed at the Sahlgrenska Academy indicates that the development of progeria in mice was inhibited upon reducing the production of this enzyme.

Related Articles


"This study is a breakthrough for our research group after years of work. When we reduce the production of the enzyme in mice, the development of all the clinical symptoms of progeria is reduced or blocked. We have also studied cultured cells from children with progeria, and can see that when the enzyme is inhibited, the growth of the cells increases by the same mechanism as in mouse cells," says Martin Bergö, Professor at the Sahlgrenska Academy, University of Gothenburg and research director at the Sahlgrenska Cancer Center.

Progeria is a rare genetic childhood disorder characterized by the appearance of accelerated aging. The classical form of progeria, called Hutchinson-Gilford Progeria Syndrome (HGPS), is caused by a spontaneous mutation, which means that it is not inherited from the parents.

Children with HGPS usually die in their teenage years from myocardial infarction and stroke.

The progeria mutation occurs in the protein prelamin A and causes it to accumulate in an inappropriate form in the membrane surrounding the nucleus. The target enzyme, called ICMT, attaches a small chemical group to one end of prelamin A. Blocking ICMT, therefore, prevents the attachment of the chemical group to prelamin A and significantly reduced the ability of the mutant protein to induce progeria.

"We are collaborating with a group in Singapore that has developed candidate ICMT inhibitor drugs and we will now test them on mice with progeria. Because the drugs have not yet been tested in humans, it will be a few years before we know whether these drugs will be appropriate for the treatment of progeria," Martin Bergö explains.

Although there are only a few hundred children in the world with progeria, the disease, children, and research have attracted a great deal of attention.

"The reason is obvious: the resemblance between progeria patients and normally-aged individuals is striking and it is tempting to speculate that progeria is a window into our normal aging process. The children develop osteoporosis, myocardial infarction, stroke, and muscle weakness. They display poor growth and lose their hair, but interestingly, they do not develop dementia or cancer," says Martin Bergö, who is also studying the impact of inhibiting ICMT on the normal aging process in mice.


Story Source:

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


Journal Reference:

  1. Mohamed X. Ibrahim, Volkan I. Sayin, Murali K. Akula, Meng Liu, Loren G. Fong, Stephen G. Young, and Martin O. Bergo. Targeting Isoprenylcysteine Methylation Ameliorates Disease in a Mouse Model of Progeria. Science, 16 May 2013 DOI: 10.1126/science.1238880

Cite This Page:

University of Gothenburg. "Accelerated aging in children: Promising treatment for progeria within reach." ScienceDaily. ScienceDaily, 16 May 2013. <www.sciencedaily.com/releases/2013/05/130516142543.htm>.
University of Gothenburg. (2013, May 16). Accelerated aging in children: Promising treatment for progeria within reach. ScienceDaily. Retrieved January 31, 2015 from www.sciencedaily.com/releases/2013/05/130516142543.htm
University of Gothenburg. "Accelerated aging in children: Promising treatment for progeria within reach." ScienceDaily. www.sciencedaily.com/releases/2013/05/130516142543.htm (accessed January 31, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Saturday, January 31, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

CDC: Get Vaccinated for Measles

CDC: Get Vaccinated for Measles

Reuters - US Online Video (Jan. 30, 2015) — The CDC is urging people to get vaccinated for measles amid an outbreak that began at Disneyland and has now infected more than 90 people. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
Obama To Outline New Plan For Personalized Medicine

Obama To Outline New Plan For Personalized Medicine

Newsy (Jan. 30, 2015) — President Obama is expected to speak with drugmakers Friday about his Precision Medicine Initiative first introduced last week. Video provided by Newsy
Powered by NewsLook.com
NFL Concussions Down; Still on Parents' Minds

NFL Concussions Down; Still on Parents' Minds

AP (Jan. 30, 2015) — The NFL announced this week that the number of game concussions dropped by a quarter over last season. Still, the dangers of the sport still weigh on players, and parents&apos; minds. (Jan. 30) Video provided by AP
Powered by NewsLook.com
U.S. Wants to Analyze DNA from 1 Million People

U.S. Wants to Analyze DNA from 1 Million People

Reuters - US Online Video (Jan. 30, 2015) — The U.S. has proposed analyzing genetic information from more than 1 million American volunteers to learn how genetic variants affect health and disease. Rough Cut (no reporter narration). Video provided by Reuters
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