Featured Research

from universities, journals, and other organizations

Abnormal Glutamine Repeats Interfere With Key Transcription Factor, Leading To Neurodegeneration

Date:
November 13, 2007
Source:
Emory University
Summary:
Although repeating sequences of three nucleotides encoding some of the bodies' 20 amino acids are a normal part of protein composition, abnormal expansion of trinucleotide repeats is the known cause of multiple inherited neurodegenerative disorders, including Huntington disease. In research with mice, scientists now have discovered more specific information about how this inherited expansion of a normal repeated DNA sequence alters gene expression.

Although repeating sequences of three nucleotides encoding some of the bodies' 20 amino acids are a normal part of protein composition, abnormal expansion of trinucleotide repeats is the known cause of multiple inherited neurodegenerative disorders, including Huntington disease.

Scientists at Emory University School of Medicine, in research with mice, now have discovered more specific information about how this inherited expansion of a normal repeated DNA sequence alters gene expression.

The inherited diseases caused by an abnormal number of glutamine repeats (generally, more than 37) are known as polyglutamine, or PolyQ diseases. The diseases lead to a progressive degeneration of nerve cells usually affecting people later in life. Although these diseases share the same abnormal expansion of the repeated glutamine sequence and some symptoms, the repeats for the different PolyQ diseases occur in genes on different chromosomes.

The scientists found that abnormal glutamine repeats interfere with the function of an essential transcription factor called TBP (TATA-box binding protein). In turn, the expanded polyQ sequence alters the interaction of TBP with other transcription factors, leading to neurodegeneration. Transcription is the process by which the genetic code in the DNA sequence is first transcribed into RNA. The RNA is subsequently translated into a protein.

"Our study has a broad impact for understanding transcriptional regulation of gene expression as well as the pathogenesis of neurodegeneration caused by expanded polyglutamine proteins," says Xiao-Jiang Li, MD, PhD, Distinguished Professor of Human Genetics in Emory University School of Medicine and the paper's senior author.

The research is reported online in the journal Nature Neuroscience.

Lead author was Meyer J. Friedman, a graduate student in Emory's Department of Human Genetics. Other Emory authors were Anjali G. Shah, Zhi-Hiu Fang, Elizabeth G. Ward, Stephen T. Warren and Shihua Li.

The research was funded by the National Institutes of Health.


Story Source:

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


Cite This Page:

Emory University. "Abnormal Glutamine Repeats Interfere With Key Transcription Factor, Leading To Neurodegeneration." ScienceDaily. ScienceDaily, 13 November 2007. <www.sciencedaily.com/releases/2007/11/071113103754.htm>.
Emory University. (2007, November 13). Abnormal Glutamine Repeats Interfere With Key Transcription Factor, Leading To Neurodegeneration. ScienceDaily. Retrieved October 21, 2014 from www.sciencedaily.com/releases/2007/11/071113103754.htm
Emory University. "Abnormal Glutamine Repeats Interfere With Key Transcription Factor, Leading To Neurodegeneration." ScienceDaily. www.sciencedaily.com/releases/2007/11/071113103754.htm (accessed October 21, 2014).

Share This



More Health & Medicine News

Tuesday, October 21, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

CDC Revamps Ebola Guidelines After Criticism

CDC Revamps Ebola Guidelines After Criticism

Newsy (Oct. 21, 2014) The Centers for Disease Control and Prevention have issued new protocols for healthcare workers interacting with Ebola patients. Video provided by Newsy
Powered by NewsLook.com
WHO: Ebola Vaccine Trials to Start a in January

WHO: Ebola Vaccine Trials to Start a in January

AP (Oct. 21, 2014) Tens of thousands of doses of experimental Ebola vaccines could be available for "real-world" testing in West Africa as soon as January as long as they are deemed safe in soon to start trials, the World Health Organization said Tuesday. (Oct. 21) Video provided by AP
Powered by NewsLook.com
First-Of-Its-Kind Treatment Gives Man Ability To Walk Again

First-Of-Its-Kind Treatment Gives Man Ability To Walk Again

Newsy (Oct. 21, 2014) A medical team has for the first time given a man the ability to walk again after transplanting cells from his brain onto his severed spinal cord. Video provided by Newsy
Powered by NewsLook.com
CDC Issues New Ebola Guidelines for Health Workers

CDC Issues New Ebola Guidelines for Health Workers

Reuters - US Online Video (Oct. 21, 2014) The U.S. Centers for Disease Control and Prevention has set up new guidelines for health workers taking care of patients infected with Ebola. Linda So 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


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