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

e 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."


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

Page: 1 2

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