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 March 4, 2015 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 March 4, 2015).

Share This


More From ScienceDaily



More Health & Medicine News

Wednesday, March 4, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Adults Only Get The Flu Twice A Decade, Researchers Say

Adults Only Get The Flu Twice A Decade, Researchers Say

Newsy (Mar. 4, 2015) Researchers found adults only get the flu about once every five years. Scientists analyzed how a person&apos;s immunity builds up over time as well. Video provided by Newsy
Powered by NewsLook.com
Mount Everest Has a Poop Problem

Mount Everest Has a Poop Problem

Buzz60 (Mar. 4, 2015) With no bathrooms to use, climbers of Mount Everest have been leaving human waste on the mountain for years, and it&apos;s becoming a health issue. Mike Janela (@mikejanela) has more. Video provided by Buzz60
Powered by NewsLook.com
Mom Triumphs Over Tragedy, Helps Other Families

Mom Triumphs Over Tragedy, Helps Other Families

AP (Mar. 3, 2015) After her son, Dax, died from a rare form of leukemia, Julie Locke decided to give back to the doctors at St. Jude Children&apos;s Research Hospital who tried to save his life. She raised $1.6M to help other patients and their families. (March 3) Video provided by AP
Powered by NewsLook.com
Looted and Leaking, South Sudan's Oil Wells Pose Health Risk

Looted and Leaking, South Sudan's Oil Wells Pose Health Risk

AFP (Mar. 3, 2015) Thick black puddles and a looted, leaking ruin are all that remain of the Thar Jath oil treatment facility, once a crucial part of South Sudan&apos;s mainstay industry. Duration: 01:13 Video provided by AFP
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