Featured Research

from universities, journals, and other organizations

Mapping The Neuron-behavior Link In Rett Syndrome

Date:
September 26, 2008
Source:
Baylor College of Medicine
Summary:
A link between certain behaviors and the lack of the protein associated with Rett Syndrome -- a devastating autism spectrum disorder -- demonstrates the importance of MeCP2 (the protein) and reveals never-before recognized functions associated with aggression and obesity, said researchers at Baylor College of Medicine in the journal Neuron.

A link between certain behaviors and the lack of the protein associated with Rett Syndrome – a devastating autism spectrum disorder – demonstrates the importance of MeCP2 (the protein) and reveals never-before recognized functions associated with aggression and obesity, said researchers at Baylor College of Medicine in a report in the current issue of the journal Neuron.

"This protein is critical for the proper function of majority of neurons," said Dr. Huda Y. Zoghbi, a BCM professor of pediatrics, neurology, neuroscience, molecular and human genetics and a Howard Hughes Medical Institute investigator who is senior author on the paper. Zoghbi and her laboratory discovered the Rett gene.

Rett Syndrome affects between 1 in 10,000 females who express a wide variety of symptoms, most of them neurological.

"Observe a girl with Rett Syndrome and you will see that she has most of the neurological symptoms you are familiar with," Zoghbi said. "There are movement problems, balance problems, tremor, seizures, autism, learning and memory problems, anxiety and even more."

Girls with Rett Syndrome lack MeCP2 (found on the X chromosome) in as many as 50 percent of their neurons. The severity and breadth of symptoms they experience depend on the percentage of neurons that lack the protein.

To determine which neurons affects which behaviors, Zoghbi and her colleagues engineered mice to lack MeCP2 in small populations of neurons in the hypothalamus, a command center of the brain that controls several physiological states.

When they eliminated the MeCP2 protein from one specific group of neurons in the hypothalamus, the animals showed one feature of Rett syndrome: increased levels of a stress hormone in response to stress. The animals also became aggressive when they were put into a novel situation.

"That meant that the few neurons that lacked MeCP2 govern this behavior," she said. "It also suggested that we need this protein to temper the response of our neurons so we behave appropriately when we experience a new social setting."

People with autism frequently get frustrated in novel situations, she said. A colleague in Germany reported seeing aggression in patients with milder Rett Syndrome.

"The other thing that surprised us was that the animals gained weight and kept on getting more obese as they got older," she said. "They ate unstoppably. In the absence of MeCP2, the signals that work within the hypothalamus to tell us we are full and need to stop eating are in part regulated by this protein. Without MeCP2, the neurons don't get the signal and you keep eating. It reveals a function of the protein not suspected before," she said.

The study also demonstrates that this technique – knocking out essential proteins in small populations of cells in the brain – could help reveal which neurons are important for which behaviors.

In the future, Zoghbi and her colleagues plan to study the function of MeCP2 in other neurons using the same technique.

Others who took part in the research include Sharyl L. Fyffe, Jeff. L. Neul, Rodney C. Samaco, Hsiao-Tuan Chao, Shay Ben-Shachar, Paolo Moretti, and Bryan E. McGill, all of BCM and Evan H. Goulding, Elinor Sullivan and Laurence H. Tecott, all of the University of California San Francisco.

Funding for this work came from the National Institutes of Health/National Institute of Neurological Disorders and Stroke, the National Institute of Child Health and Human Development Mental Retardation and Developmental Disabilities Research Center, the International Rett Syndrome Foundation, Autism Speaks and the Simons Foundation.


Story Source:

The above story is based on materials provided by Baylor College of Medicine. Note: Materials may be edited for content and length.


Cite This Page:

Baylor College of Medicine. "Mapping The Neuron-behavior Link In Rett Syndrome." ScienceDaily. ScienceDaily, 26 September 2008. <www.sciencedaily.com/releases/2008/09/080924124542.htm>.
Baylor College of Medicine. (2008, September 26). Mapping The Neuron-behavior Link In Rett Syndrome. ScienceDaily. Retrieved October 21, 2014 from www.sciencedaily.com/releases/2008/09/080924124542.htm
Baylor College of Medicine. "Mapping The Neuron-behavior Link In Rett Syndrome." ScienceDaily. www.sciencedaily.com/releases/2008/09/080924124542.htm (accessed October 21, 2014).

Share This



More Mind & Brain News

Tuesday, October 21, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Your Birth Season Might Determine Your Temperament

Your Birth Season Might Determine Your Temperament

Newsy (Oct. 20, 2014) A new study says the season you're born in can determine your temperament — and one season has a surprising outcome. Video provided by Newsy
Powered by NewsLook.com
Court Ruling Means Kids' Online Activity Could Be On Parents

Court Ruling Means Kids' Online Activity Could Be On Parents

Newsy (Oct. 17, 2014) In a ruling attorneys for both sides agreed was a first of its kind, a Georgia appeals court said parents can be held liable for what kids put online. Video provided by Newsy
Powered by NewsLook.com
The Best Foods To Boost Your Mood

The Best Foods To Boost Your Mood

Buzz60 (Oct. 17, 2014) Feeling down? Reach for the refrigerator, not the medicine cabinet! TC Newman (@PurpleTCNewman) shares some of the best foods to boost your mood. Video provided by Buzz60
Powered by NewsLook.com
You Can Get Addicted To Google Glass, Apparently

You Can Get Addicted To Google Glass, Apparently

Newsy (Oct. 15, 2014) Researchers claim they’ve diagnosed the first example of the disorder in a 31-year-old U.S. Navy serviceman. 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