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:1/28/2016)... SYNA ), a leading developer of human interface solutions, today ... --> --> Net revenue ... to the comparable quarter last year to $470.5 million. Net income ... $0.93 per diluted share. --> ... 2016 grew 9 percent over the prior year period to $60.3 ...
(Date:1/22/2016)... January 22, 2016 ... addition of the  "Global Behavioral Biometric ... --> http://www.researchandmarkets.com/research/4lmf2s/global_behavioral ) has ... Behavioral Biometric Market 2016-2020"  report to ... and Markets ( http://www.researchandmarkets.com/research/4lmf2s/global_behavioral ) has announced ...
(Date:1/20/2016)... , Jan. 20, 2016   MedNet Solutions , ... entire spectrum of clinical research, is pleased to announce ... significant achievements are the result of the company,s laser ... iMedNet eClinical , it,s comprehensive, easy-to-use and highly ... --> Key MedNet growth achievements in 2015 ...
Breaking Biology News(10 mins):
(Date:2/5/2016)... Amarantus BioScience Holdings, Inc. ... developing products for Regenerative Medicine, Neurology and Orphan Diseases, ... (RPDD) from the US Food and Drug Administration (FDA) ... previously granted orphan drug designation (ODD) by the US ... Holdings, Inc. (OTCQB: AMBS), a biotechnology ...
(Date:2/4/2016)... New FDA action date of July 22, 2016   ... 22, 2016   --> - New FDA ... - Lifitegrast has the potential to be the only product approved ... and symptoms of dry eye disease in adults --> - ... the U.S. in the past decade indicated for the treatment of signs and ...
(Date:2/4/2016)... Church, VA (PRWEB) , ... ... ... first-year cybersecurity conference presented by Bloomsburg University’s Digital Forensics Club, takes place ... PA. The two-day event features 20+ speakers and activities such as workshops ...
(Date:2/4/2016)... MENLO PARK, Calif. , Feb. 4, 2016 ... the "Company"), a biopharmaceutical company focused on the development and ... present at the 18 th Annual BIO CEO ... 10:00 a.m. EST in New York, NY ... CEO, will provide an update on the ongoing clinical trial ...
Breaking Biology Technology: