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:6/22/2016)... ANGELES , June 22, 2016 /PRNewswire/ ... identity management and verification solutions, has partnered ... edge software solutions for Visitor Management, Self-Service ... provides products that add functional enhancements ... partnership provides corporations and venues with an ...
(Date:6/15/2016)... New York , June 15, 2016 /PRNewswire/ ... a new market report titled "Gesture Recognition Market by ... and Forecast, 2016 - 2024". According to the report, ... USD 11.60 billion in 2015 and is estimated ... reach USD 48.56 billion by 2024.  ...
(Date:6/2/2016)... LONDON , June 2, 2016 ... has awarded the 44 million US Dollar project, ... Security Embossed Vehicle Plates including Personalization, Enrolment, and IT Infrastructure ... world leader in the production and implementation of Identity Management ... in January, however Decatur was selected ...
Breaking Biology News(10 mins):
(Date:6/23/2016)... ... June 23, 2016 , ... STACS DNA Inc., the ... at the Arkansas State Crime Laboratory, has joined STACS DNA as a Field Application ... team,” said Jocelyn Tremblay, President and COO of STACS DNA. “In further expanding our ...
(Date:6/23/2016)... June 23, 2016 Apellis Pharmaceuticals, Inc. ... clinical trials of its complement C3 inhibitor, APL-2. ... multiple ascending dose studies designed to assess the ... subcutaneous injection in healthy adult volunteers. ... as a single dose (ranging from 45 to ...
(Date:6/23/2016)... 2016 Andrew D ... http://doi.org/10.17925/OHR.2016.12.01.22 Published recently in ... from touchONCOLOGY, Andrew D Zelenetz , discusses ... care is placing an increasing burden on healthcare ... therapies. With the patents on many biologics expiring, ...
(Date:6/23/2016)... ... June 23, 2016 , ... ClinCapture, the only free validated ... will showcase its product’s latest features from June 26 to June 30, 2016 ... on Disrupting Clinical Trials in The Cloud during the conference. DIA (Drug ...
Breaking Biology Technology: