Navigation Links
Neural tissue contains imbalanced levels of proteins, U-M study finds
Date:6/2/2010

ANN ARBOR, Mich. 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 populationswhen 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.


'/>"/>

Contact: Jenna Frye
jennafry@umich.edu
7-340-764-2220
University of Michigan Health System
Source:Eurekalert

Related biology news :

1. Induced neural stem cells: Not quite ready for prime time
2. Brain-controlled cursor doubles as a neural workout
3. Case Western Reserve University discovers Merkel cell originates from skin, not the neural crest
4. Mouse brain rewires its neural circuits to recuperate from damaged neural function after stroke
5. Essential nutrient found in eggs may help lower risk of neural tube defects
6. Neural stem cell differentiation factor discovered
7. Identification of a key molecular pathway required for brain neural circuit formation
8. Early brain activity sheds new light on the neural basis of reading
9. Creating ideal neural cells for clinical use
10. Low levels of vitamin B12 may increase risk for neural tube defects
11. Ex vivo neural stem cell expansion
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:3/23/2017)... Research and Markets has announced the addition of the ... Forecast to 2025" report to their offering. ... The Global Vehicle Anti-Theft System ... over the next decade to reach approximately $14.21 billion by 2025. ... forecasts for all the given segments on global as well as ...
(Date:3/20/2017)... , March 20, 2017 PMD Healthcare ... personal spirometer and Wellness Management System (WMS), a remote, ... Founded in 2010, PMD Healthcare is a Medical Device, ... a mission dedicated to creating innovative solutions that empower ... With that intent focus, PMD developed the first ever ...
(Date:3/7/2017)... BRIGHTON, England , March 7, 2017 Brandwatch ... been chosen by The Prince,s Trust to uncover insights ... insights across The Trust. The UK,s leading youth charity ... track social campaign results and get a better understanding of the ... ...
Breaking Biology News(10 mins):
(Date:3/24/2017)... Biotech Ltd. ("Sinovac" or the "Company") (NASDAQ: SVA), a leading provider ... that its board of directors has amended its shareholder rights plan. The ... 2017 to March 27, 2018. The amendment was not in response to ... ... Ltd. is a China -based biopharmaceutical company that ...
(Date:3/23/2017)... ... ... to announce it has become the premiere team-building cooking event company in San Diego. ... as Illumina, HP and Qualcomm, and is ranked #1 in its category on Trip Advisor. ... new team building format, a way for teams to not only interact with one another ...
(Date:3/23/2017)... MENLO PARK, Calif., March 23, 2017  BioPharmX ... developing products for the dermatology market, today reported ... Jan. 31, 2017, and will provide an update ... from the year. "We are pleased ... productive year for BioPharmX," said President Anja Krammer. ...
(Date:3/23/2017)... Colo. , March 23, 2017  Agriculture technology ... Series A financing and note conversion to commercialize its ... Planet is focused on developing products that are simultaneously ... $30 million in the last 18 months. This latest ... North Bridge Venture Partners. The company,s ...
Breaking Biology Technology: