Featured Research

from universities, journals, and other organizations

Protecting the brain from Huntington's disease

Date:
February 23, 2010
Source:
University of Western Ontario
Summary:
Huntington's disease (HD) is a cruel, hereditary condition that leads to physical and mental deterioration and eventually, death. HD sufferers are born with the disease although they don't show symptoms until late in life. In a new study, researchers identified a protective pathway in the brain that may explain why symptoms take so long to appear.

Huntington's disease (HD) is a cruel, hereditary condition that leads to severe physical and mental deterioration, psychiatric problems and eventually, death. Currently, there are no treatments to slow down or stop it. HD sufferers are born with the disease although they do not show symptoms until late in life.

Related Articles


In a new study published in The Journal of Neuroscience, Stephen Ferguson and Fabiola Ribeiro of Robarts Research Institute at The University of Western Ontario identified a protective pathway in the brain that may explain why HD symptoms take so long to appear. The findings could also lead to new treatments for HD.

The symptoms of Huntington's disease are caused by cell death in specific regions of the brain. Patients who have HD are born with a mutated version of the protein huntingtin (Htt), which is thought to cause these toxic effects. While researchers know HD results from this single, mutated protein, no one seems to know exactly what it does, why it does not cause symptoms until later in life, or why it kills a specific set of brain cells, even though Htt is found in every single cell in the human body.

Ferguson and Ribeiro used a genetically-modified mouse model of HD to look at the effects of mutated Htt on the brain. "We found there was some kind of compensation going on early in the life of these mice that was helping to protect them from the development of the disease," says Ferguson, director of the Molecular Brain Research Group at Robarts, and a professor in the Department of Physiology & Pharmacology at Western's Schulich School of Medicine and Dentistry. "As they age, they lose this compensation and the associated protective effects, which could explain the late onset of the disease."

Ferguson adds that metabotropic glutamate receptors (mGluRs), which are responsible for communication between brain cells, play an important role in these protective effects. By interacting with the mutant Htt protein, mGluRs change the way the brain signals in the early stages of HD in an attempt to offset the disease, and save the brain from cell death. As a result, mGluRs could offer a drug target for HD treatment.

Because HD is a dominant genetic disease, every child with an affected parent has a 50 per cent chance of inheriting the fatal condition. This research, funded by the Canadian Institutes of Health Research, sheds light on the onset of HD and the potential role of a mutant protein in patients, paving the way for the development of new drug therapies.


Story Source:

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


Cite This Page:

University of Western Ontario. "Protecting the brain from Huntington's disease." ScienceDaily. ScienceDaily, 23 February 2010. <www.sciencedaily.com/releases/2010/02/100223101428.htm>.
University of Western Ontario. (2010, February 23). Protecting the brain from Huntington's disease. ScienceDaily. Retrieved January 26, 2015 from www.sciencedaily.com/releases/2010/02/100223101428.htm
University of Western Ontario. "Protecting the brain from Huntington's disease." ScienceDaily. www.sciencedaily.com/releases/2010/02/100223101428.htm (accessed January 26, 2015).

Share This


More From ScienceDaily



More Mind & Brain News

Monday, January 26, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

How Technology Is Ruining Snow Days For Students

How Technology Is Ruining Snow Days For Students

Newsy (Jan. 25, 2015) — More schools are using online classes to keep from losing time to snow days, but it only works if students have Internet access at home. Video provided by Newsy
Powered by NewsLook.com
Weird Things Couples Do When They Lose Their Phone

Weird Things Couples Do When They Lose Their Phone

BuzzFeed (Jan. 24, 2015) — Did you back it up? Do you even know how to do that? Video provided by BuzzFeed
Powered by NewsLook.com
Smart Wristband to Shock Away Bad Habits

Smart Wristband to Shock Away Bad Habits

Reuters - Innovations Video Online (Jan. 23, 2015) — A Boston start-up is developing a wristband they say will help users break bad habits by jolting them with an electric shock. Ben Gruber reports. Video provided by Reuters
Powered by NewsLook.com
Amazing Technology Allows Blind Mother to See Her Newborn Son

Amazing Technology Allows Blind Mother to See Her Newborn Son

RightThisMinute (Jan. 23, 2015) — Not only is Kathy seeing her newborn son for the first time, but this is actually the first time she has ever seen a baby. Kathy and her sister, Yvonne, have been legally blind since childhood, but thanks to an amazing new technology, eSight glasses, which gives those who are legally blind the ability to see, she got the chance to see the birth of her son. It&apos;s an incredible moment and an even better story. Video provided by RightThisMinute
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