Featured Research

from universities, journals, and other organizations

Molecular Basis For Neurodegeneration In Ataxia Telangiectasia

Date:
April 14, 2008
Source:
Cold Spring Harbor Laboratory
Summary:
New research lends insight into the pathogenesis of neurodegeneration in Ataxia telangiectasia. Ataxia telangiectasia (A-T) is a rare, genetic immunodeficiency disease that affects multiple organ systems and is characterized by neurodegeneration and cancer predisposition. A-T is caused by recessive mutations in the ataxia telangiectasia mutated (ATM) gene.

An upcoming paper from Dr. David Wassarman (University of Wisconsin School of Medicine and Public Health) in the May 1 issue of Genes & Development lends new insight into the pathogenesis of neurodegeneration in Ataxia telangiectasia.

Related Articles


Ataxia telangiectasia (A-T) is a rare, genetic immunodeficiency disease that affects multiple organ systems and is characterized by neurodegeneration and cancer predisposition. A-T is caused by recessive mutations in the ataxia telangiectasia mutated (ATM) gene.

While existing animal models have established how ATM mutations contribute to genomic instability and cancer susceptibility, Dr. Wassarman's paper reveals how ATM mutations cause neurodegeneration.

The scientists generated a Drosophila model of A-T, in which neurodeneneration occurs in the absence of induced DNA damage -- as it does in human A-T patients. Thus, Dr. Wassarman's model most faithfully recapitulates neurodegeneration associated with the human disease.

Using this model, Dr. Wassarman and colleagues determined ATM functions normally to prevent neurons from re-entering the cell cycle.

"At the end of the day, ATM-dependent arrest of cell growth is critical for both neuron function and tumor suppression," explains Dr. Wassarman. Furthermore, he is excited by the findings, as they "point to possible therapeutic potential of CDC25 and other cell cycle inhibitors" to treat A-T as well as other neurodegenerative disorders.


Story Source:

The above story is based on materials provided by Cold Spring Harbor Laboratory. Note: Materials may be edited for content and length.


Cite This Page:

Cold Spring Harbor Laboratory. "Molecular Basis For Neurodegeneration In Ataxia Telangiectasia." ScienceDaily. ScienceDaily, 14 April 2008. <www.sciencedaily.com/releases/2008/04/080414145641.htm>.
Cold Spring Harbor Laboratory. (2008, April 14). Molecular Basis For Neurodegeneration In Ataxia Telangiectasia. ScienceDaily. Retrieved November 27, 2014 from www.sciencedaily.com/releases/2008/04/080414145641.htm
Cold Spring Harbor Laboratory. "Molecular Basis For Neurodegeneration In Ataxia Telangiectasia." ScienceDaily. www.sciencedaily.com/releases/2008/04/080414145641.htm (accessed November 27, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Thursday, November 27, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Ebola Leaves Orphans Alone in Sierra Leone

Ebola Leaves Orphans Alone in Sierra Leone

AFP (Nov. 27, 2014) — The Ebola epidemic sweeping Sierra Leone is having a profound effect on the country's children, many of whom have been left without any family members to support them. Duration: 01:02 Video provided by AFP
Powered by NewsLook.com
Experimental Ebola Vaccine Shows Promise In Human Trial

Experimental Ebola Vaccine Shows Promise In Human Trial

Newsy (Nov. 27, 2014) — A recent test of a prototype Ebola vaccine generated an immune response to the disease in subjects. Video provided by Newsy
Powered by NewsLook.com
Pet Dogs to Be Used in Anti-Ageing Trial

Pet Dogs to Be Used in Anti-Ageing Trial

Reuters - Innovations Video Online (Nov. 26, 2014) — Researchers in the United States are preparing to discover whether a drug commonly used in human organ transplants can extend the lifespan and health quality of pet dogs. Video provided by Reuters
Powered by NewsLook.com
Today's Prostheses Are More Capable Than Ever

Today's Prostheses Are More Capable Than Ever

Newsy (Nov. 26, 2014) — Advances in prosthetics are making replacement body parts stronger and more lifelike than they’ve ever been. 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