Featured Research

from universities, journals, and other organizations

Huntington's Disease Problem Start Early

Date:
January 10, 2008
Source:
Baylor College of Medicine
Summary:
The damaging effects of the mutated protein involved in Huntington's disease take place earlier in cell life than previously believed.

Working with fruit flies, researchers have discovered a new mechanism by which the abnormal protein in Huntington's disease causes neurodegeneration. They have also manipulated the flies to successfully suppress that neurodegeneration, which they said suggests potential treatments to delay the onset and progression of the disease in humans.

Related Articles


The damaging effects of the mutated protein involved in Huntington's disease take place earlier in cell life than previously believed, said researchers from Baylor College of Medicine in Houston in a new report.

"This research provides evidence of toxicity by huntingtin (the protein involved in the disease) early during the disease process," said Dr. Juan Botas, associate professor of molecular and human genetics at BCM. Previously, researchers thought that the protein, which is extremely large, begins its negative effects after it is cut and imported into the cell's nucleus. However, Botas and his colleagues showed that the toxic effects are felt even before the protein is cleaved.

"Early in the disease, the full-length protein already causes neurotransmission problems at the level of the synapse," he said. The synapse allows communication between two cells using chemicals called neurotransmitters. "We investigated the nature of those neurotransmitter defects, and at the same time, identified the genes that could ameliorate those defects."

Huntington's disease is caused by a mutation in the gene for the huntingtin protein (htt) that causes a genetic "stutter"--an abnormally long number of repeats of the amino acid glutamine at one end of the protein. Huntington's disease is one of nine diseases associated with a repeat of the three nucleotides [C (cytosine) A (adenine) G (guanine)]. The combination of the three make an amino acid called glutamine. When the CAG repeats an inordinate amount of time, it results in a disease. Collectively, the disorders are called polyglutamine diseases.

Among those genes that can compensate for the misfire at the synapse are those that govern calcium channels (tiny pores in the cell's membrane that allow the in- and out-flow of calcium), said Botas. These genes and others involved in synaptic transmission could serve as targets for the development of potential drug therapies.

This research appears in the January 10 edition of the journal Neuron.

Others who took part in this work include Eliana Romero, Guang-Ho Cha, Patrik Verstreken, Cindy V. Ly and Hugo J. Bellen, all of BCM, and Robert Hughes of the Buck Institute in Novato, California. Funding for this research comes from the National Institute of Neurological Disorders and Stroke, the Korea Science and Engineering Foundation, the Huntington's Disease Foundation of America and the Ruth L. Kirschstein National Research Service Award.


Story Source:

The above story is based on materials provided by Baylor College of Medicine. Note: Materials may be edited for content and length.


Cite This Page:

Baylor College of Medicine. "Huntington's Disease Problem Start Early." ScienceDaily. ScienceDaily, 10 January 2008. <www.sciencedaily.com/releases/2008/01/080109173827.htm>.
Baylor College of Medicine. (2008, January 10). Huntington's Disease Problem Start Early. ScienceDaily. Retrieved December 18, 2014 from www.sciencedaily.com/releases/2008/01/080109173827.htm
Baylor College of Medicine. "Huntington's Disease Problem Start Early." ScienceDaily. www.sciencedaily.com/releases/2008/01/080109173827.htm (accessed December 18, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Thursday, December 18, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Kids Die While Under Protective Services

Kids Die While Under Protective Services

AP (Dec. 18, 2014) As part of a six-month investigation of child maltreatment deaths, the AP found that hundreds of deaths from horrific abuse and neglect could have been prevented. AP's Haven Daley reports. (Dec. 18) Video provided by AP
Powered by NewsLook.com
UN: Up to One Million Facing Hunger in Ebola-Hit Countries

UN: Up to One Million Facing Hunger in Ebola-Hit Countries

AFP (Dec. 17, 2014) Border closures, quarantines and crop losses in West African nations battling the Ebola virus could lead to as many as one million people going hungry, UN food agencies said on Wednesday. Duration: 00:52 Video provided by AFP
Powered by NewsLook.com
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
Why Your Boss Should Let You Sleep In

Why Your Boss Should Let You Sleep In

Newsy (Dec. 17, 2014) According to research out of the University of Pennsylvania, waking up for work is the biggest factor that causes Americans to lose sleep. 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