Featured Research

from universities, journals, and other organizations

Why certain diseases affect specific organs: Neural tissue contains imbalanced levels of proteins, study finds

Date:
June 3, 2010
Source:
University of Michigan Health System
Summary:
Why do some diseases affect only specific organs, leaving others invulnerable? Researchers have found neural tissue contains imbalanced levels of proteins, which may explain the brain's susceptibility to a debilitating childhood movement disorder. Compared to nonneuronal cells, neurons have dramatically lower levels of torsinB, a sister protein that can work similarly to torsinA. When the DYT1 gene mutation causes a defect in torsinA, torsinB can take over its role in all other cells except for neuronal cells.

Why do some diseases affect only specific organs, leaving others invulnerable? Researchers from the University of Michigan have found neural tissue contains imbalanced levels of proteins, which may explain the brain's susceptibility to a debilitating childhood movement disorder.

Known as DYT1 dystonia, the disease causes involuntary twisting and repetitive movements or abnormal postures. It's caused by a mutation in the DYT1 gene, which contains instructions for making the torsinA protein, causing this protein become defective. The defective protein causes disruption in an area of the brain that controls movement, leading to the abnormal movements.

"We want to understand why dystonia affects only brain cells in order to treat children," says William Dauer, M.D., Elinor Levine associate professor in the department of neurology at the University of Michigan.

Previously, Dauer and his colleagues used genetic engineering to create the same mutation in mouse DNA that causes the disease in humans. These mice had a neural specific defect similar to the brain-specific abnormality in human patients with dystonia.

The researchers used this mouse model to determine why neurons were affected, while the rest of the body was unaffected. They found that compared to nonneuronal cells, neurons have dramatically lower levels of torsinB, a sister protein that can work similarly to torsinA. When the DYT1 gene mutation causes a defect in torsinA, torsinB can take over its role in all other cells except for neuronal cells.

In fact, when Dauer and his colleagues experimentally reduced the amount of torsinB in skin cells with the DYT1 mutation, they developed abnormalities just like those found in neurons, says Dauer. "This could explain why one organ may be selectively affected in other diseases. The cells in that organ might lack proteins that help them to withstand certain genetic mutations or environmental insults."

"I compare this to populations -- when during flu season only people with weakened immune systems get sick, while others don't. It's really quite simple," he says.

In order to treat dystonia, Dauer says further research will continue to discover the specific groups of movement-controlling neurons in the brain that are most affected by the defective torsinA protein.

Ultimately, research is needed to discover how to modulate the proteins in cells to protect the vulnerable cells from disease.

"The overall goal is to find treatment for this disease," Dauer says.

Additional authors: Along with Dauer, Connie M. Kim, M.D., of Columbia University and Alex Perez, Guy Perkins, Ph.D., and Mark H. Ellisman, Ph.D., of the University of California.

Funding: This work was funded by the Dystonia Medical Research Foundation, the Bachmann Strauss Foundation, the Parkinson's Foundation and the National Institute of Health.


Story Source:

The above story is based on materials provided by University of Michigan Health System. The original article was written by Jenna Frye. Note: Materials may be edited for content and length.


Journal Reference:

  1. C. E. Kim, A. Perez, G. Perkins, M. H. Ellisman, W. T. Dauer. A molecular mechanism underlying the neural-specific defect in torsinA mutant mice. Proceedings of the National Academy of Sciences, 2010; 107 (21): 9861 DOI: 10.1073/pnas.0912877107

Cite This Page:

University of Michigan Health System. "Why certain diseases affect specific organs: Neural tissue contains imbalanced levels of proteins, study finds." ScienceDaily. ScienceDaily, 3 June 2010. <www.sciencedaily.com/releases/2010/06/100602121210.htm>.
University of Michigan Health System. (2010, June 3). Why certain diseases affect specific organs: Neural tissue contains imbalanced levels of proteins, study finds. ScienceDaily. Retrieved September 2, 2014 from www.sciencedaily.com/releases/2010/06/100602121210.htm
University of Michigan Health System. "Why certain diseases affect specific organs: Neural tissue contains imbalanced levels of proteins, study finds." ScienceDaily. www.sciencedaily.com/releases/2010/06/100602121210.htm (accessed September 2, 2014).

Share This




More Mind & Brain News

Tuesday, September 2, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Can You Train Your Brain To Eat Healthy?

Can You Train Your Brain To Eat Healthy?

Newsy (Sep. 1, 2014) New research says if you condition yourself to eat healthy foods, eventually you'll crave them instead of junk food. Video provided by Newsy
Powered by NewsLook.com
Coffee Then Napping: The (New) Key To Alertness

Coffee Then Napping: The (New) Key To Alertness

Newsy (Aug. 30, 2014) Researchers say having a cup of coffee then taking a nap is more effective than a nap or coffee alone. Video provided by Newsy
Powered by NewsLook.com
Young Entrepreneurs Get $100,000, If They Quit School

Young Entrepreneurs Get $100,000, If They Quit School

AFP (Aug. 29, 2014) Twenty college-age students are getting 100,000 dollars from a Silicon Valley leader and a chance to live in San Francisco in order to work on the start-up project of their dreams, but they have to quit school first. Duration: 02:20 Video provided by AFP
Powered by NewsLook.com
Baby Babbling Might Lead To Faster Language Development

Baby Babbling Might Lead To Faster Language Development

Newsy (Aug. 29, 2014) A new study suggests babies develop language skills more quickly if their parents imitate the babies' sounds and expressions and talk to them often. 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