Featured Research

from universities, journals, and other organizations

New Therapeutic Insight Into Duchenne Muscular Dystrophy

Date:
April 2, 2007
Source:
Cold Spring Harbor Laboratory
Summary:
Medical researchers identify a key genetic component of and possible therapeutic target for Duchenne muscular dystrophy.

In the April 1st issue of Genes & Development, Dr. Bruce Spiegelman (Dana Farber Cancer Institute) and colleagues identify a key genetic component of and possible therapeutic target for Duchenne muscular dystrophy.

Related Articles


Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy, affecting about 1 in 3000 males each year. It is an X-linked recessive disease, in which mutations in the dystrophin gene causes progressive and degenerative muscle weakness. DMD is generally lethal by age 30.

Dr. Spiegelman and colleagues found that a protein called PGC-1alpha regulates the point of connection between the end of a motor neuron and a muscle fiber -- what researchers call the "neuromuscular junction." Electrical impulses travel through the neuromuscular junction, ultimately causing the muscle to contract. Previous research has shown that PGC-1alpha expression is induced by physical exercise and motor neuron activity, and mediates the anti-atrophic effects of nerve activity on muscle mass.

Dr. Spiegelman and colleagues analyzed the function of PGC-1alpha in a mouse model of DMD. They found that PGC-1alpha activates the expression of several genes that are aberrantly inactivated in DMD. In fact, by inducing PGC-1alpha expression in these transgenic mice, the scientists were able to improve DMD symptoms.

"These data clearly show that experimental elevation of PGC-1 alpha has therapeutic promise in an animal model of Duchene's muscular dystrophy. We hope this will lead eventually to therapeutics for a terrible disease for which there is no effective treatment at the present time," explains Dr. Spiegelman.


Story Source:

The above story is based on materials provided by Cold Spring Harbor Laboratory. Note: Materials may be edited for content and length.


Cite This Page:

Cold Spring Harbor Laboratory. "New Therapeutic Insight Into Duchenne Muscular Dystrophy." ScienceDaily. ScienceDaily, 2 April 2007. <www.sciencedaily.com/releases/2007/04/070402102412.htm>.
Cold Spring Harbor Laboratory. (2007, April 2). New Therapeutic Insight Into Duchenne Muscular Dystrophy. ScienceDaily. Retrieved February 27, 2015 from www.sciencedaily.com/releases/2007/04/070402102412.htm
Cold Spring Harbor Laboratory. "New Therapeutic Insight Into Duchenne Muscular Dystrophy." ScienceDaily. www.sciencedaily.com/releases/2007/04/070402102412.htm (accessed February 27, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Friday, February 27, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Sleeping Too Much Or Too Little Might Increase Stroke Risk

Sleeping Too Much Or Too Little Might Increase Stroke Risk

Newsy (Feb. 26, 2015) People who sleep more than eight hours per night are 45 percent more likely to have a stroke, according to a University of Cambridge study. Video provided by Newsy
Powered by NewsLook.com
London Show Dissects History of Forensic Science

London Show Dissects History of Forensic Science

AFP (Feb. 25, 2015) Forensic science, which has fascinated generations with its unravelling of gruesome crime mysteries, is being put under the microscope in an exhibition of real criminal investigations in London. Duration: 00:53 Video provided by AFP
Powered by NewsLook.com
Michigan Couple Celebrates Identical Triplets

Michigan Couple Celebrates Identical Triplets

AP (Feb. 25, 2015) A suburban Detroit couple who have two older children are adjusting to life after becoming parents to identical triplets _ a multiple birth a doctor calls rare. (Feb. 25) Video provided by AP
Powered by NewsLook.com
Mayor Says District of Columbia to Go Ahead With Pot Legalization

Mayor Says District of Columbia to Go Ahead With Pot Legalization

Reuters - News Video Online (Feb. 25, 2015) Washington&apos;s mayor says the District of Columbia will move forward with marijuana legalization, despite pushback from Congress. 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