Featured Research

from universities, journals, and other organizations

Singled Out: Spotting Mutant Neurons In Normal Brains Offers Clues To Fragile X

Date:
November 2, 2004
Source:
Cell Press
Summary:
Using an animal model system, researchers have advanced our understanding of Fragile X Syndrome by successfully visualizing individual mutant neurons in an otherwise normal brain. They find that brain neurons that fail to express appropriate levels of Fragile X protein are structurally abnormal and make defective connections to other neurons.

Using an animal model system, researchers have advanced our understanding of Fragile X Syndrome by successfully visualizing individual mutant neurons in an otherwise normal brain. They find that brain neurons that fail to express appropriate levels of Fragile X protein are structurally abnormal and make defective connections to other neurons. The work is reported by a team led by Kendal Broadie at Vanderbilt University.

Fragile X Syndrome is the most common type of inherited mental retardation. Since the early 1990's, it has been known that the disease results from the loss of a single gene and, for the last several years, that the Fragile X gene product regulates the expression of other proteins. However, the link between this molecular function and the Fragile X brain defect has remained a mystery.

Employing a fruit fly model of the disease, the researchers utilized a new technique that allows the generation of single mutant nerve cells that are marked by their expression of a glowing fluorescent protein, making these nerve cells distinctly visible in an otherwise normal brain. In their experiments, the researchers were able to observe mutant nerve cells that either completely lacked the Fragile X protein or overexpressed it, the fluorescent protein label allowing the entire architecture of these neurons to be visualized in the intact brain.

From the observations made with this approach, it became apparent that the Fragile X protein acts as a so-called "global negative regulator" of nerve cell complexity. Nerve cells lacking the protein are more complex; they exhibit more processes, more branching, and more growth. Conversely, nerve cells overexpressing the protein are simplified; they lack normal levels of processes, branching, and growth. Moreover, electron microscope imaging revealed that removal of the Fragile X protein dramatically impairs the ultrastructure of the synaptic connections that are formed by the mutant cells. Taken together, these findings suggest that Fragile X Syndrome may be caused by alteration in the growth of the brain's nerve cells and the neuronal connectivity between these cells.

###

Luyuan Pan, Yong Q. Zhang, Elvin Woodruff, and Kendal Broadie: "The Drosophila Fragile X Gene Negatively Regulates Neuronal Elaboration and Synaptic Differentiation"

Publishing in Current Biology, Vol. 14, Issue 20, October 26, 2004, pages 1863–1870.


Story Source:

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


Cite This Page:

Cell Press. "Singled Out: Spotting Mutant Neurons In Normal Brains Offers Clues To Fragile X." ScienceDaily. ScienceDaily, 2 November 2004. <www.sciencedaily.com/releases/2004/10/041030151930.htm>.
Cell Press. (2004, November 2). Singled Out: Spotting Mutant Neurons In Normal Brains Offers Clues To Fragile X. ScienceDaily. Retrieved August 30, 2014 from www.sciencedaily.com/releases/2004/10/041030151930.htm
Cell Press. "Singled Out: Spotting Mutant Neurons In Normal Brains Offers Clues To Fragile X." ScienceDaily. www.sciencedaily.com/releases/2004/10/041030151930.htm (accessed August 30, 2014).

Share This




More Health & Medicine News

Saturday, August 30, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

We've Got Mites Living In Our Faces And So Do You

We've Got Mites Living In Our Faces And So Do You

Newsy (Aug. 30, 2014) A new study suggests 100 percent of adult humans (those over 18 years of age) have Demodex mites living in their faces. Video provided by Newsy
Powered by NewsLook.com
Liberia Continues Fight Against Ebola

Liberia Continues Fight Against Ebola

AFP (Aug. 30, 2014) Authorities in Liberia try to stem the spread of the Ebola epidemic by raising awareness and setting up sanitation units for people to wash their hands. Duration: 00:41 Video provided by AFP
Powered by NewsLook.com
California Passes 'yes-Means-Yes' Campus Sexual Assault Bill

California Passes 'yes-Means-Yes' Campus Sexual Assault Bill

Reuters - US Online Video (Aug. 30, 2014) California lawmakers pass a bill requiring universities to adopt "affirmative consent" language in their definitions of consensual sex, part of a nationwide drive to curb sexual assault on campuses. Linda So reports. Video provided by Reuters
Powered by NewsLook.com
3 Things To Know About The Ebola Outbreak's Progression

3 Things To Know About The Ebola Outbreak's Progression

Newsy (Aug. 29, 2014) Here are three things you need to know about the deadly Ebola outbreak's progression this week. 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:
from the past week

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