Navigation Links
UT scientist uncovers trigger to fatal neurodegenerative disease
Date:6/22/2011

Jeremy Smith, Governor's Chair for Molecular Biophysics at the University of Tennessee, Knoxville, has helped reveal a key trigger of GerstmannStrusslerScheinker (GSS) syndrome, a rare but deadly neurodegenerative disease. The finding could have far-reaching implications for the treatment of other neurodegenerative diseases such as Alzheimer's, Huntington's, and Parkinson's.

Smith conducted his research with two collaborators in Italy: Isabella Daidone, a former postdoctoral researcher of his who is now at the University of L'Aquila, and Alfredo Di Nola of the University of Rome "La Sapienza."

Most GSS patients begin developing symptoms in their late fifties. Symptoms include loss of memory, difficulty speaking, and unsteadiness and lead to progressive dementia, and then death within a few months or years. There is presently no cure or treatment. The disease results from a single, tiny mutation in a protein, resulting in it having a wrong shapethrough "misfolding"then aggregating to form amyloid plaques in the brain.

"Ever since the 'mad cow' scare in Britain in the 1990s, which led to several hundred human deaths and 4.4 million cattle being destroyed, I've been interested in finding out more about these fascinating diseases of wrongly-shaped proteins," said Smith, who was born in England.

The team compared high-performance computer simulations of the structures of the normal and the GSSmutant proteins. They found the GSS protein looks dramatically different from the normal form and revealed how its shape is primed for plaque formation.

"This research shows how computer simulation can be used to pinpoint changes in molecular structure that lead directly to disease," said Smith. "We think that a similar line of investigation should prove beneficial in understanding the origins of other amyloid diseases such as Alzheimer's, Parkinson's, and rheumatoid arthritis. Once the origin is understood at molecular detail, strategies to rationally prevent and cure a disease can be conceived."

The findings can be found in the article, "Molecular Origin of GerstmannStrusslerScheinker Syndrome: Insight from Computer Simulation of an Amyloidogenic Prion Peptide" in this month's edition of the Biophysical Journal.


'/>"/>

Contact: Whitney Holmes
wholmes7@utk.edu
865-974-5460
University of Tennessee at Knoxville
Source:Eurekalert

Related biology news :

1. Jefferson scientists deliver toxic genes to effectively kill pancreatic cancer cells
2. Scientist plans to test for blood pressure genes affected by age
3. Scientists identify novel inhibitor of human microRNA
4. Argonne scientists peer into heart of compound that may detect chemical, biological weapons
5. MU scientists go green with gold, distribute environmentally friendly nanoparticles
6. Scientists identify gene that may contribute to improved rice yield
7. Scientists discover why a mothers high-fat diet contributes to obesity in her children
8. MU scientists see how HIV matures into an infection
9. Earth scientists keep an eye on Texas
10. Thinking it through: Scientists call for policy to guide biofuels industry toward sustainability
11. Scientists identify a molecule that coordinates the movement of cells
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:10/4/2017)... Oct. 4, 2017  GCE Solutions, a global clinical research organization ... document anonymization solution on October 4, 2017. Shadow is designed to ... comply with policy 0070 of the European Medicines Agency (EMA) in ... ... ...
(Date:7/20/2017)... Delta (NYSE: DAL ) customers now can use ... Reagan Washington National Airport (DCA). ... Delta launches biometrics to board aircraft at Reagan Washington ... Delta,s biometric boarding pass experience that ... integrated into the boarding process to allow eligible Delta SkyMiles Members who ...
(Date:6/14/2017)...  IBM (NYSE: IBM ) is introducing several innovative ... to developing collaboration between startups and global businesses, taking place ... the event, nine startups will showcase the solutions they have ... France is one ... a 30 percent increase in the number of startups created ...
Breaking Biology News(10 mins):
(Date:10/7/2017)... ... October 06, 2017 , ... ... its ProxiMeta™ Hi-C metagenome deconvolution product, featuring the first commercially available Hi-C ... software to perform Hi-C metagenome deconvolution using their own facilities, supplementing the ...
(Date:10/6/2017)... (PRWEB) , ... October 06, 2017 , ... ... entrepreneurship within the healthcare and technology sector at their fourth annual Conference where ... featuring 30 inspiring speakers and the ELEVATE pitch competition showcasing early stage digital ...
(Date:10/5/2017)... , ... October 05, 2017 , ... LabRoots , ... scientists from around the world, is giving back to cancer research with a month-long ... , Now through October 31, shoppers can use promo code PinkRibbon to get ...
(Date:10/5/2017)... ... October 05, 2017 , ... NIH ... of its Nanobind DNA/RNA extraction technology . Nanobind is a novel magnetic ... surface and that can be used for a wide variety of sample preparation ...
Breaking Biology Technology: