Featured Research

from universities, journals, and other organizations

A role for glia in the progression of Rett syndrome

Date:
June 29, 2011
Source:
The Rett Syndrome Research Trust
Summary:
New research shows that glia play a key role in preventing the progression of the most prominent Rett Syndrome symptoms displayed by mouse models of the disease: lethality, irregular breathing and apneas, hypoactivity and decreased dendritic complexity.

A paper published online June 29 in Nature reveals that glia play a key role in preventing the progression of the most prominent Rett Syndrome symptoms displayed by mouse models of the disease: lethality, irregular breathing and apneas, hypoactivity and decreased dendritic complexity. The discovery, funded in part by the Rett Syndrome Research Trust (RSRT) was led by Gail Mandel, Ph.D., an investigator of the Howard Hughes Medical Institute at Oregon Health and Science University.

Related Articles


Rett Syndrome, the most physically disabling of the autism spectrum disorders, is caused by mutations in the methyl CpG-binding protein (MeCP2). Rett Syndrome strikes little girls almost exclusively, with first symptoms usually appearing before the age of 18 months. These children lose speech, motor control and functional hand use, and many suffer from seizures, orthopedic and severe digestive problems, breathing and other autonomic impairments. Most live into adulthood, and require total, round- the- clock care. There are several mouse models for RTT in which MeCP2 has been deleted: these mice accurately recapitulate many of the human symptoms.

In a seminal 2007 Science paper, Adrian Bird and colleagues from the University of Edinburgh showed that global re-expression of MeCP2 in mouse models dramatically reversed Rett symptoms, even in very late-stage disease. This led to the idea that the damage to neurons in RTT was reversible.

In 2009, Mandel and collaborator Nurit Ballas (Stony Brook University) showed that the MeCP2 protein was present in all types of glia. Glial cells are composed of astrocytes, oligodendrocytes and microglia. Glia support and interact with neurons in innumerable ways, from providing the structural underpinnings and guidance of axons and dendrites (the neuronal processes that carry information), to creating protective insulation for axons, to providing energy substrates necessary for neuronal function. Until the reports of MeCP2's presence in glia, Rett Syndrome was thought to be caused exclusively by MeCP2 deficiencies in neurons.

Mandel and Ballas and colleagues now show that in a mouse model of Rett Syndrome, re-expression of MeCP2 solely in astrocytes, in male mice at 4 weeks of age and in female mice between the ages of 5 to 7 months, rescues the lifespan, breathing, anxiety, and locomotor activities associated with the global knockout mice.

Mandel states: "The RTT plot now thickens…we need to think about contributions from multiple cell types to this disease. The idea that neurons and glia might serve different roles in Rett Syndrome, in initiation and progression of symptoms, is reminiscent of the situation in another neurological disorder, an inherited form of ALS. Thus, a role for glia may turn out to be a more common theme in many neurological diseases. It will be important to determine if other glial types play roles in RTT, and to further investigate how normal neurons and astrocytes interact, a currently active but controversial area."

First author Daniel Lioy (Howard Hughes Medical Institute, OHSU) comments, "Future studies will focus on trying to identify the key molecules in astrocytes that might mediate the rescue. These molecules may provide new avenues for targeted pharmacological intervention for Rett."

"This new and unexpected result by the Mandel lab reveals important clues regarding the function of MeCP2 and how its absence causes devastation. The Rett Syndrome Research Trust will continue to support high-level exploration with the conviction that understanding how this protein works will open new doors to treatment approaches," said Monica Coenraads, Executive Director of RSRT and mother to a daughter with Rett Syndrome.


Story Source:

The above story is based on materials provided by The Rett Syndrome Research Trust. Note: Materials may be edited for content and length.


Journal Reference:

  1. Daniel T. Lioy, Saurabh K. Garg, Caitlin E. Monaghan, Jacob Raber, Kevin D. Foust, Brian K. Kaspar, Petra G. Hirrlinger, Frank Kirchhoff, John M. Bissonnette, Nurit Ballas, Gail Mandel. A role for glia in the progression of Rett’s syndrome. Nature, 2011; DOI: 10.1038/nature10214

Cite This Page:

The Rett Syndrome Research Trust. "A role for glia in the progression of Rett syndrome." ScienceDaily. ScienceDaily, 29 June 2011. <www.sciencedaily.com/releases/2011/06/110629171224.htm>.
The Rett Syndrome Research Trust. (2011, June 29). A role for glia in the progression of Rett syndrome. ScienceDaily. Retrieved November 23, 2014 from www.sciencedaily.com/releases/2011/06/110629171224.htm
The Rett Syndrome Research Trust. "A role for glia in the progression of Rett syndrome." ScienceDaily. www.sciencedaily.com/releases/2011/06/110629171224.htm (accessed November 23, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Sunday, November 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Ebola-Hit Sierra Leone's Late Cocoa Leaves Bitter Taste

Ebola-Hit Sierra Leone's Late Cocoa Leaves Bitter Taste

AFP (Nov. 23, 2014) The arable district of Kenema in Sierra Leone -- at the centre of the Ebola outbreak in May -- has been under quarantine for three months as the cocoa harvest comes in. Duration: 01:32 Video provided by AFP
Powered by NewsLook.com
Don't Fall For Flu Shot Myths

Don't Fall For Flu Shot Myths

Newsy (Nov. 23, 2014) Misconceptions abound when it comes to your annual flu shot. Medical experts say most people older than 6 months should get the shot. Video provided by Newsy
Powered by NewsLook.com
WFP: Ebola Risks Heightened Among Women Throughout Africa

WFP: Ebola Risks Heightened Among Women Throughout Africa

AFP (Nov. 21, 2014) Having children has always been a frightening prospect in Sierra Leone, the world's most dangerous place to give birth, but Ebola has presented an alarming new threat for expectant mothers. Duration: 00:37 Video provided by AFP
Powered by NewsLook.com
Could Your Genes Be The Reason You're Single?

Could Your Genes Be The Reason You're Single?

Newsy (Nov. 21, 2014) Researchers in Beijing discovered a gene called 5-HTA1, and carriers are reportedly 20 percent more likely to be single. 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