Featured Research

from universities, journals, and other organizations

Drug Protects Brain Cells In Huntington's Disease Model, Researchers Find

Date:
July 27, 2007
Source:
UT Southwestern Medical Center
Summary:
A drug used in some countries to treat the symptoms of Huntington's disease prevents death of brain cells in mice genetically engineered to mimic the hereditary condition, researchers have found. The research sheds light on the biochemical mechanisms involved in the disease and suggests new avenues of study for preventing brain-cell death in at-risk people before symptoms appear.

Dr. Ilya Bezprozvanny, associate professor of physiology, led research showing that the drug tetrabenazine -- used in some countries to treat the symptoms of Huntington's disease -- prevents death of brain cells in mice genetically engineered to mimic the hereditary condition.
Credit: UT Southwestern Medical Center

A drug used in some countries to treat the symptoms of Huntington's disease prevents death of brain cells in mice genetically engineered to mimic the hereditary condition, UT Southwestern Medical Center researchers have found.

Related Articles


The research sheds light on the biochemical mechanisms involved in the disease and suggests new avenues of study for preventing brain-cell death in at-risk people before symptoms appear.

"The drug can actually prevent brain cells from dying," said Dr. Ilya Bezprozvanny, associate professor of physiology at UT Southwestern. "It's much more important than people thought."

The study, of which Dr. Bezprozvanny is senior author, appears in the July 25 issue of The Journal of Neuroscience.

The drug, called tetrabenazine (TBZ), is commercially distributed as Xenazine or Nitoman and blocks the action of dopamine, a compound that some nerve cells use to signal others. TBZ is approved for use in several countries, but not the U.S., to treat uncontrollable muscle movements in Huntington's and other neurological conditions.

Huntington's is a fatal genetic condition that usually manifests around ages 30 to 45, according to the Huntington's Disease Society of America. About one in 10,000 people in America have the disease, with another 200,000 at risk. One of the most famous people with Huntington's was folk singer Woody Guthrie, who died in 1967.

Huntington's is caused by a dominant gene, meaning that a person carrying the gene is certain to develop the disease and has a 50 percent chance of passing it on to his or her children. Symptoms include jerky, uncontrollable movements called chorea and deterioration of reasoning abilities and personality. Symptoms begin after many brain cells have already died.

Although a genetic test exists, some people with a family history of Huntington's choose not to be tested because there is no cure and because they fear loss of health insurance. There are treatments to lessen the symptoms, but there is currently no way to slow or halt the progression of the disease.

In the current study, the UT Southwestern researchers used mice that were genetically engineered to carry the mutant human gene for Huntington's, causing symptoms and death of brain cells similar to those seen in the disease. The study focused on an area of the brain called the striatum, which plays a critical role in relaying signals concerning motion and higher thought and receives signals from several brain regions.

The striatum is primarily made up of nerve cells called medium spiny neurons, which undergo widespread death in Huntington's. One major input to the striatum comes from an area called the substantia nigra, which controls voluntary movements and sends signals to the striatum via nerve cells that release dopamine.

The researchers conducted various coordination tests on both normal and genetically manipulated mice. Engineered mice given a drug that increased brain dopamine levels performed worse on these tasks, while TBZ protected against this effect. Most importantly, TBZ appears to reduce significantly cell loss in the striatum of the engineered mice, the scientists report.

"More research is needed to determine whether this protective effect might also be present in humans, and also whether at-risk people would benefit from the drug," Dr. Bezprozvanny said.

Clinical trials in humans would be very difficult, however, because trials require many participants and there is no easy way to score effectiveness of a presymptomatic drug, Dr. Bezprozvanny said. Thus, his future studies in animals will look at the effectiveness of TBZ given just after initial symptoms have developed. This situation simulates what would probably happen in a human trial, he said.

Other UT Southwestern researchers involved in the study were Dr. Tie-Shan Tang, instructor in physiology; and Dr. Xi Chen and Dr. Jing Liu, postdoctoral researchers in physiology.

The work was supported by the Robert A. Welch Foundation, the Huntington's Disease Society of America, the Hereditary Disease Foundation, the HighQ Foundation and the National Institute of Neurological Disorders and Stroke.


Story Source:

The above story is based on materials provided by UT Southwestern Medical Center. Note: Materials may be edited for content and length.


Cite This Page:

UT Southwestern Medical Center. "Drug Protects Brain Cells In Huntington's Disease Model, Researchers Find." ScienceDaily. ScienceDaily, 27 July 2007. <www.sciencedaily.com/releases/2007/07/070725093635.htm>.
UT Southwestern Medical Center. (2007, July 27). Drug Protects Brain Cells In Huntington's Disease Model, Researchers Find. ScienceDaily. Retrieved April 18, 2015 from www.sciencedaily.com/releases/2007/07/070725093635.htm
UT Southwestern Medical Center. "Drug Protects Brain Cells In Huntington's Disease Model, Researchers Find." ScienceDaily. www.sciencedaily.com/releases/2007/07/070725093635.htm (accessed April 18, 2015).

Share This


More From ScienceDaily



More Mind & Brain News

Saturday, April 18, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Our Love Of Puppy Dog Eyes Explained By Science

Our Love Of Puppy Dog Eyes Explained By Science

Newsy (Apr. 17, 2015) Researchers found a spike in oxytocin occurs in both humans and dogs when they gaze into each other&apos;s eyes. Video provided by Newsy
Powered by NewsLook.com
Scientists Find Link Between Gestational Diabetes And Autism

Scientists Find Link Between Gestational Diabetes And Autism

Newsy (Apr. 17, 2015) Researchers who analyzed data from over 300,000 kids and their mothers say they&apos;ve found a link between gestational diabetes and autism. Video provided by Newsy
Powered by NewsLook.com
Video Messages Help Reassure Dementia Patients

Video Messages Help Reassure Dementia Patients

AP (Apr. 17, 2015) Family members are prerecording messages as part of a unique pilot program at the Hebrew Home in New York. The videos are trying to help victims of Alzheimer&apos;s disease and other forms of dementia break through the morning fog of forgetfulness. (April 17) Video provided by AP
Powered by NewsLook.com
Common Pain Reliever Might Dull Your Emotions

Common Pain Reliever Might Dull Your Emotions

Newsy (Apr. 16, 2015) Each week, millions of Americans take acetaminophen to dull minor aches and pains. Now researchers say it might blunt life&apos;s highs and lows, too. Video provided by Newsy
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