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.

"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 October 23, 2014 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 October 23, 2014).

Share This



More Health & Medicine News

Thursday, October 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Orthodontist Mom Jennifer Salzer on the Best Time for Braces

Orthodontist Mom Jennifer Salzer on the Best Time for Braces

Working Mother (Oct. 22, 2014) — Is your child ready? Video provided by Working Mother
Powered by NewsLook.com
U.S. Issues Ebola Travel Restrictions, Are Visa Bans Next?

U.S. Issues Ebola Travel Restrictions, Are Visa Bans Next?

Newsy (Oct. 22, 2014) — Now that the U.S. is restricting travel from West Africa, some are dropping questions about a travel ban and instead asking about visa bans. Video provided by Newsy
Powered by NewsLook.com
US to Track Everyone Coming from Ebola Nations

US to Track Everyone Coming from Ebola Nations

AP (Oct. 22, 2014) — Stepping up their vigilance against Ebola, federal authorities said Wednesday that everyone traveling into the US from Ebola-stricken nations will be monitored for symptoms for 21 days. (Oct. 22) Video provided by AP
Powered by NewsLook.com
Doctors Help Paralysed Man Walk Again, Patient in Disbelief

Doctors Help Paralysed Man Walk Again, Patient in Disbelief

AFP (Oct. 22, 2014) — Polish doctors describe how they helped a paralysed man walk again, with the patient in disbelief at the return of sensation to his legs. Duration: 1:04 Video provided by AFP
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