Navigation Links
Scientists identify new protein in the neurological disorder dystonia
Date:5/6/2014

MANHATTAN, Kan. A collaborative discovery involving Kansas State University researchers may lead to the first universal treatment for dystonia, a neurological disorder that affects nearly half a million Americans.

Michal Zolkiewski, associate professor of biochemistry and molecular biophysics at Kansas State University, and Jeffrey Brodsky at the University at Pittsburgh co-led a study that focused on a mutated protein associated with early onset torsion dystonia, or EOTD, the most severe type of dystonia that typically affects adolescents before the age of 20. Dystonia causes involuntary and sustained muscle contractions that can lead to paralysis and abnormal postures.

"It's a painful and debilitating disease for which there is no cure or treatment that would be effective for all patients," Zolkiewski said. "There are some treatments that are being tested, but nothing is really available to those patients that would cure the symptoms completely."

In addition to Zolkiewski and Brodsky, researchers involved in the study included Hui-Chuan Wu, Kansas State University doctoral student in biochemistry and molecular biophysics, Taiwan, and colleagues at the University of Texas Southwestern Medical Center and the University of Adelaide in Australia.

The Journal of Biological Chemistry recently published the team's study, "The BiP molecular chaperone plays multiple roles during the biogenesis of TorsinA, a AAA+ ATPase associated with the neurological disease Early-Onset Torsion Dystonia." The study was funded by the Dystonia Medical Research Foundation.

Researchers built the study on a decade-old discovery that patients with early onset torsion dystonia typically have a mutated gene that encodes the protein TorsionA.

"TorsinA is a protein that all people have in their bodies," Zolkiewski said. "It appears to perform an important role in the nervous system, but currently nobody knows what that role is. There also is no understanding of the link between the mutation and dystonia."

In order to study protein expression in a living organism, researchers used yeast one of the simplest living systems. The yeast was engineered to produce the human protein TorsionA.

Observations revealed that a second protein named BiP pronounced "dip" helps process the TorsinA protein and maintain its active form. Additionally, researchers found that BiP also guides TorsinA to being destroyed by cells if the protein is defective. Humans carry the BiP protein as well as the TorsinA protein.

"BiP is a molecular chaperone that assists other proteins in maintaining their function," Zolkiewski said. "In this study we found that BiP really has a dual role. On one hand it's helping TorsinA and on the other it's leading to its degradation."

Future studies may focus on BiP as a target for treating dystonia, as modulating BiP in human cells would affect TorsinA, Zolkiewski said.

"Because we don't know what exactly the function of TorsinA is, we may not be able to design a treatment based on that protein," Zolkiewski said. "We know what BiP does, however. It is a pretty well-studied chaperone, which makes it much easier to work with."


'/>"/>

Contact: Michal Zolkiewski
michalz@k-state.edu
785-532-3083
Kansas State University
Source:Eurekalert

Related medicine news :

1. Scientists awarded grant to develop diagnostics for cancer, rheumatoid arthritis, colitis
2. Norwegian neuroscientists elected to National Academy of Sciences
3. NIH scientists establish monkey model of hantavirus disease
4. Stanford scientists create circuit board modeled on the human brain
5. A civil war inside our cells: Scientists show how our bodies fight off jumping genes
6. Scientists find way to target cells resistant to chemo
7. Scientists identify cancer specific cell for potential treatment of gastric cancer
8. Scientists pinpoint protein that could improve small cell lung cancer therapies
9. AMP publishes curriculum recommendations for medical laboratory scientists
10. Scientists alter fat metabolism in animals to prevent most common type of heart disease
11. Scientists discover a new way to enhance nerve growth following injury
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:5/24/2016)... ... ... New Brunswick, New Jersey: This year marks Children’s Specialized Hospital ’s 125th ... the anniversary, the hospital has themed the milestone “Hats Off” and kicked off the ... on Saturday, May 21, at Johnson Park in Piscataway, New Jersey. , ...
(Date:5/24/2016)... — , ... (PRWEB) ... of three new members of its Advisory Board. Joining the ... Sarah Kusch. “All three of them embody the mission of our ... are very fortunate to have them as we continue to expand ...
(Date:5/24/2016)... ... ... Boyd Industries, a leading supplier of dental chairs and cabinetry , will ... dentistry , at AAPD 2016, the annual conference and trade show of the American ... keeps dental hand pieces and other anxiety-provoking pieces of the dental delivery system out ...
(Date:5/24/2016)... Angeles, CA (PRWEB) , ... May 24, 2016 ... ... to everything egg freezing, today announced the official relaunch of its community and ... use their frozen eggs. Eggsurance's mission is to create a safe and welcoming ...
(Date:5/24/2016)... Maryland (PRWEB) , ... May 24, 2016 , ... ... announced the appointment of Jonathan (Jon) Otterstatter to its board of ... is a proven leader in the development of technological innovations that lead to ...
Breaking Medicine News(10 mins):
(Date:5/23/2016)... , May 23, 2016 ... Size, Share, Development, Growth and Demand Forecast to ... and Other), by Application (Drug Discovery and Development, ... Users (Pharmaceuticals, Life Science and Biotechnology, Academic and ... Market Research, the global mass spectrometry market ...
(Date:5/23/2016)... , May 23, 2016 Non-invasive ... detection of multiple diseases; ,Technology to be presented at ... Yissum Research Development Company of the Hebrew University ... a research agreement with Aurum Ventures MKI, the technology investment ... of a new diagnostic approach for early detection of ...
(Date:5/23/2016)... -- The global  reprocessed medical devices market ... according to a new study by Grand View Research, ... the lack of centralized support for waste disposal in ... for reprocessed medical devices market. Additionally, the long-term cost-efficiency ... the original device is the high impact rendering driver ...
Breaking Medicine Technology: