Navigation Links
Neural tissue contains imbalanced levels of proteins, U-M study finds

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
University of Michigan Health System

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:
(Date:11/12/2015)... -- A golden retriever that stayed healthy despite having the ... a new lead for treating this muscle-wasting disorder, report ... MIT and Harvard and the University of São Paolo ... Cell, pinpoints a protective gene that boosts muscle ... Boston Children,s lab of Lou Kunkel , PhD, ...
(Date:11/10/2015)... 10, 2015 About signature ... helps to identify and verify the identity of ... as the secure and accurate method of authentication ... particular individual because each individual,s signature is highly ... when dynamic signature of an individual is compared ...
(Date:10/29/2015)... Oct. 29, 2015   MedNet Solutions , an ... spectrum of clinical research, is pleased to announce that ... Association (MHTA) as one of only three finalists for ... – Small and Growing" category. The Tekne Awards honor ... shown superior technology innovation and leadership. ...
Breaking Biology News(10 mins):
(Date:12/1/2015)... ALEXANDRIA, Va. , Dec. 1, 2015 ... provides senior debt to life sciences and healthcare services ... senior secured term loan with MDRejuvena, Inc. ("the Company"). ... commercialization and continued development of the Company,s Rejuvaphyl™ and ... --> Rejuvaphyl is the MDRejuvena brand of high ...
(Date:12/1/2015)... , ... December 01, 2015 , ... ... announces Park NX10 SICM Module, an add-on scanning ion conductance microscopy module to ... of SICM to an AFM. , Park SICM benefits virtually all materials characterization ...
(Date:12/1/2015)... , Dec. 1, 2015 Cepheid (Nasdaq: ... its participation at the Piper Jaffray Healthcare Conference in ... morning, the Company is reaffirming its outlook for the ... 2016, in addition to discussing longer term business model ... Chief Executive Officer.  "We continue to be the fastest ...
(Date:12/1/2015)... , December 1, 2015 ... addition of the  "2016 U.K. Virology and ... Forecasts for 100 Tests, Supplier Shares by ...  report to their offering.  --> ... the  "2016 U.K. Virology and Bacteriology Testing ...
Breaking Biology Technology: