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 22, 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 22, 2014).

Share This


More From ScienceDaily



More Plants & Animals News

Monday, December 22, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Christmas Kissing Good for Health

Christmas Kissing Good for Health

Reuters - Innovations Video Online (Dec. 22, 2014) Scientists in Amsterdam say couples transfer tens of millions of microbes when they kiss, encouraging healthy exposure to bacteria. Suzannah Butcher reports. Video provided by Reuters
Powered by NewsLook.com
Brain-Dwelling Tapeworm Reveals Genetic Secrets

Brain-Dwelling Tapeworm Reveals Genetic Secrets

Reuters - Innovations Video Online (Dec. 22, 2014) Cambridge scientists have unravelled the genetic code of a rare tapeworm that lived inside a patient's brain for at least four year. Researchers hope it will present new opportunities to diagnose and treat this invasive parasite. Matthew Stock reports. Video provided by Reuters
Powered by NewsLook.com
New Fish Species Discovered, Setting Record for World's Deepest

New Fish Species Discovered, Setting Record for World's Deepest

Buzz60 (Dec. 22, 2014) A new species of fish is discovered living five miles beneath the ocean surface, making it the deepest living fish on earth. Jen Markham has the story. Video provided by Buzz60
Powered by NewsLook.com
Earthworms Provide Cancer-Fighting Bacteria

Earthworms Provide Cancer-Fighting Bacteria

Reuters - Innovations Video Online (Dec. 21, 2014) Polish scientists isolate bacteria from earthworm intestines which they say may be used in antibiotics and cancer treatments. Suzannah Butcher reports. 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


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