Featured Research

from universities, journals, and other organizations

Mice holding back muscular dystrophy research?

Date:
December 4, 2009
Source:
BioMed Central
Summary:
Humans and mice have previously unknown and potentially critical differences in one of the genes responsible for Duchenne muscular dystrophy. Researchers have found that two major features of a key DMD gene are present in most mammals, including humans, but are specifically absent in mice and rats, calling into question the use of the mouse as the principal model animal for studying DMD.

Humans and mice have previously unknown and potentially critical differences in one of the genes responsible for Duchenne muscular dystrophy (DMD). Researchers writing in the open access journal BMC Biology have found that two major features of a key DMD gene are present in most mammals, including humans, but are specifically absent in mice and rats, calling into question the use of the mouse as the principal model animal for studying DMD.

Related Articles


Roland Roberts led a team of researchers from King's College London, UK, and was funded by the Muscular Dystrophy Campaign. The team made the discovery while studying α-dystrobrevin, a component of the dystrophin protein complex that is disordered in DMD. Roberts said, "Two previously unrecognized features (a gene switch or promoter and a novel binding site for the adaptor protein syntrophin) are encoded by the α-dystrobrevin gene of almost all four-legged animals except mice. We assume that this tardy recognition of key features of a gene that has been intensively studied since its discovery 13 years ago is due to the predominance of the mouse as the model organism for studying DMD and the specific destruction of these parts of the gene in the mouse."

A major consequence of these findings is that mice (and their rat and hamster relatives) are likely to be particularly poor models in which to study the effects of DMD on the brain. Roberts added, "The brain is the major site of α-dystrobrevin expression and we now know that the mouse is missing more than 50% of the brain α-dystrobrevins. The fact that there are fundamental differences between the brains of mice and humans potentially limits our understanding of the role of dystrobrevins and DMD-related complexes in this organ. In fact, almost all of our knowledge of the function of α-dystrobrevin has been gleaned from the mouse."

DMD is a fatal skeletal myopathy, causing loss of muscle tissue throughout the body. It is also associated with substantial neurological effects including learning difficulties, night blindness, defective color vision and a suggestion of personality disorders, so studying the mechanisms in the brain underlying these effects is crucial.


Story Source:

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


Journal Reference:

  1. Sabrina V Boehm, Panayiotis Constantinou, Sipin Tan, Hong Jin and Roland G Roberts. Profound human/mouse differences in alpha-dystrobrevin isoforms: a novel syntrophin-binding site and promoter missing in mouse and rat. BMC Biology, (in press) [link]

Cite This Page:

BioMed Central. "Mice holding back muscular dystrophy research?." ScienceDaily. ScienceDaily, 4 December 2009. <www.sciencedaily.com/releases/2009/12/091203222134.htm>.
BioMed Central. (2009, December 4). Mice holding back muscular dystrophy research?. ScienceDaily. Retrieved December 17, 2014 from www.sciencedaily.com/releases/2009/12/091203222134.htm
BioMed Central. "Mice holding back muscular dystrophy research?." ScienceDaily. www.sciencedaily.com/releases/2009/12/091203222134.htm (accessed December 17, 2014).

Share This


More From ScienceDaily



More Plants & Animals News

Wednesday, December 17, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

When You Lose Weight, This Is Where The Fat Goes

When You Lose Weight, This Is Where The Fat Goes

Newsy (Dec. 17, 2014) Can fat disappear into thin air? New research finds that during weight loss, over 80 percent of a person's fat molecules escape through the lungs. Video provided by Newsy
Powered by NewsLook.com
Rover Finds More Clues About Possible Life On Mars

Rover Finds More Clues About Possible Life On Mars

Newsy (Dec. 17, 2014) NASA's Curiosity rover detected methane on Mars and organic compounds on the surface, but it doesn't quite prove there was life ... yet. Video provided by Newsy
Powered by NewsLook.com
Ivory Trade Boom Swamps Law Efforts

Ivory Trade Boom Swamps Law Efforts

Reuters - Business Video Online (Dec. 17, 2014) Demand for ivory has claimed the lives of tens of thousands of African elephants and now a conservation report says the illegal trade is overwhelming efforts to enforce the law. Amy Pollock reports. Video provided by Reuters
Powered by NewsLook.com
Uphill Battle to Tackle Indonesian Shark Fishing

Uphill Battle to Tackle Indonesian Shark Fishing

AFP (Dec. 17, 2014) Sharks are hauled ashore every day at a busy market on the central Indonesian island of Lombok, the hub of a booming trade that provides a livelihood for local fishermen but is increasingly alarming environmentalists. Duration: 00:42 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


Plants & Animals

Earth & Climate

Fossils & Ruins

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