Navigation Links
Prion discovery could help keep deadly brain diseases in check
Date:1/16/2014

New research from David Westaway, PhD, of the University of Alberta and Jiri Safar, PhD, Case Western Reserve University School of Medicine has uncovered a quality control mechanism in brain cells that may help keep deadly neurological diseases in check for months or years.

The findings, published in The Journal of Clinical Investigation, "present a breakthrough in understanding the secret life of prion molecules in the brain and may offer a new way to treat prion diseases," said Westaway, Director of the Centre for Prions and Protein Folding Diseases and Professor of Neurology in the Faculty of Medicine and Dentistry at the University of Alberta.

Prion diseases lead to incurable neurodegenerative disorders such as Creutzfeldt-Jakob disease in humans, mad cow disease (Bovine Spongiform Encephalopathy) and chronic wasting disease in deer and elk. The diseases are caused by the conversion of normal cellular prion proteins into the diseased form.

For years, scientists have been perplexed by two unexplained characteristics of prion infections: vastly differing asymptomatic periods lasting up to five decades and when symptoms do arise, greatly varying accumulation of the diseased proteins. In striking contrast, test tube prions replicate rapidly, and in a matter of days reach levels found in brains in the final stage of the disease.

"Our study investigated the molecular mechanism of this intriguing puzzle," said Safar, Co-Director of the National Prion Disease Pathology Surveillance Center and Associate Professor in Departments of Pathology and Neurology in Case Western Reserve University School of Medicine.

In probing these mysteries, Westaway, Safar, their teams and other collaborating researchers in the U.S., Italy and the Netherlands studied a molecule called the 'shadow of the prion protein.'

"Dramatic changes in this shadow protein led us to expand our view to include the normal prion protein itself," said Westaway. "This is a crucial molecule in brain cells because it is pirated as the raw material to make diseased prion proteins."

The production and degradation of the normal prion protein had previously received little attention because it was assumed its production pipeline did not vary.

"The puzzle of the long asymptomatic time period required sorting out the different types of prion protein molecules. Our laboratory developed new techniques to tease out these subtle differences in shape," Safar said.

The researchers discovered a marked drop in the amount of the normal prion protein in eight different types of prion diseases. Strikingly, this drop occurred months or years before the animal models showed tell-tale clinical symptoms of the brain disease.

"Our belief is that cells under prion attack are smarter than we once thought," Westaway said. "They not only sense the molecular piracy by the diseased proteins, but they also adopt a simple and at least partly effective protective response they minimize the amount raw material from the pipeline for prion production."

"We believe we can kill two birds with one stone, because the normal prion protein is also a receptor for toxicity. Augmenting this natural protective response may be a preferred route to cure prion infections," Safar added.

The study's discovery of a natural protective response can also explain the long latency period in other more common neurodegenerative diseases.

"The pre-clinical phase of the diseasebefore it shows symptomsis when you want to set things straight. We may be able to take a slow disease and bring it to a complete standstill," Westaway said. "Since some scientists believe the normal prion protein is an accessory in the brain cell death of Alzheimer's disease, gaining a new understanding of rare yet lethal prion diseases may provoke fresh insights into human dementias."


'/>"/>
Contact: Bev Betkowski
bev.betkowski@ualberta.ca
780-492-3808
University of Alberta
Source:Eurekalert

Related medicine news :

1. Prion-like proteins drive several diseases of aging
2. Normal prion protein regulates iron metabolism
3. Prions and cancer: A story unfolding
4. Prions in the brain eliminated by homing molecules
5. Autism Learning Games: Camp Discovery App Utilizes Technology to Revolutionize Learning for Children with Autism
6. Discovery to Facilitate Eradication of Polio Worldwide
7. Cell division discovery could offer fresh insight into cancer
8. Expert Project Manager Joins TERIS, a National Leader in eDiscovery, Information Governance, Computer Forensics and Relativity
9. Transvaginal Mesh Lawsuit News: Bernstein Liebhard LLP Notes Issuance of New Order Pertaining to Discovery in Federal Boston Scientific Litigation
10. Judge Overseeing Federal Biomet Hip Lawsuits Won't Name Special Discovery Master, Bernstein Liebhard LLP Reports
11. Ovarian cancer discovery deepens knowledge of survival outcomes
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:12/4/2016)... ... December 03, 2016 , ... The Lymphoma Research Foundation (LRF) ... and serving the lymphoma community through a comprehensive series of education programs, outreach ... in New York City, with long-time partners The Paul Foundation, on November 10, ...
(Date:12/4/2016)... ... December 03, 2016 , ... Penrose Senior Care Auditors® was ... Tuesday evening at the 26th Annual SMU Cox Dallas 100™ Awards Ceremony and ... Institute for Entrepreneurship. Dallas 100™, co-founded by the Caruth Institute, honors the ingenuity, ...
(Date:12/4/2016)... ... December 04, 2016 , ... Responsible dental care hinges on regular brushing ... achieve optimal results. This important necessity inspired an inventor from Las Vegas, Nev., to ... ensure that people break or avoid bad techniques of brushing the teeth in order ...
(Date:12/2/2016)... ... December 02, 2016 , ... On Dec. 2, 2016, CURE® magazine ... San Diego honoring the 2016 MPN Heroes—eight individuals who have made a difference in ... and beyond the standard of care, demonstrating leadership within the MPN community and/or a ...
(Date:12/2/2016)... , ... December 02, 2016 , ... ... announces the launch of its 60-day free trial program for all of the ... make the offer a truly hassle free experience. , FlexiSpot’s unique desktop risers ...
Breaking Medicine News(10 mins):
(Date:12/4/2016)... highly innovative, personalized cell-based treatment for a high-risk form of the most ... Children,s Hospital of Philadelphia today reported new results ... The physician-scientists presented findings at the annual meeting of the ... Continue Reading ... ...
(Date:12/2/2016)... 2, 2016 bioLytical Laboratories, un líder mundial en test ... INSTI HIV Self Test , a los miembros de la Kenya Pharmaceutical Association. ... ... INSTI HIV Self Test! (PRNewsFoto/bioLytical Laboratories) ...      (Photo: http://photos.prnewswire.com/prnh/20161201/444905 ) bioLytical fue invitada por ...
(Date:12/2/2016)... , Dec 2, 2016 Research and Markets ... Share, Development, Growth and Demand Forecast to 2022" report to their ... , , ... $6 billion in 2015, and it is expected to grow at a ... is expected to witness faster growth during the forecast period, a CAGR ...
Breaking Medicine Technology: