Prion disease may be caused by buildup of cellular trash, say Stanford researchers

STANFORD, Calif. - Mutant mice whose brains gradually become peppered with small holes resembling those found in prion disease lack a protein involved in disposing of cellular trash, say researchers at Stanford University Medical Center. The finding may shed light on how diseases such as Creutzfeldt-Jakob, bovine spongiform encephalopathy (also known as mad cow disease), and scrapie wreak havoc in humans and other mammals, and lends further support to the growing notion that glitches in protein turnover may be the unifying element in many neurodegenerative disorders.

Although the scientists caution that the mutant mice don't accumulate the misfolded protein, or prion, associated with the infectious forms of spongiform encephalopathy, the rodents' brains are dead-ringers for the brains of people and cows who have died from the disease. They speculate that the mutation in a ubiquitin ligase - a protein that flags other proteins for destruction by the cell's recycling machinery - may represent a downstream step in the cascade of events that leaves the brain looking somewhat like a kitchen sponge.

"No one really understands how or why spongy degeneration develops," said Gregory Barsh, MD, PhD, professor of pediatrics and of genetics in the Stanford School of Medicine, in whose laboratory the research was conducted. "Now we have a molecular handle with which to study it." The research is published in the Jan. 31 issue of Science.

Ubiquitin ligases are just a few members of a complex team of proteins that make up the ubiquitin pathway. Together they identify and physically tag abnormal, misfolded or simply worn-out proteins for dismantling in the cell's recycling center. Until recently they were about as glamorous as garbage collectors.

The study of neurodegenerative diseases such as Parkinson's, Huntington's and Lou Gehrig's upped the ubiquitin ante, however, with the discovery that patients' brain cells share a common trait: large clumps

Contact: Krista Conger
Stanford University Medical Center

Page: 1 2 3

Related biology news :

1. Prion propagation: Avoiding the toxic oligomer
2. Prions act as stepping stones in evolution
3. Prions offer nanotech building tool
4. Prions may play crucial role in evolution.
5. Science: Prion proteins may signal trouble -- literally
6. Prions are modular
7. New Evidence On How Prions Turn Fatal
8. University Of California-San Francisco Researchers Report Test That Detects Prion Diseases, Illuminates Novel Findings About Infectious Prions
9. Progress In Prion Research: Three-Dimensional Structure Of Prion Protein Fully Uncovered
10. Schepens Eye Research Institute receives Roadmap grant to develop center for curing eye diseases
11. Alzheimers disease is not accelerated aging

Post Your Comments:

(Date:7/22/2020)... ... July 21, 2020 , ... USDM ... technology and compliance, announces a new solution to manage regulated workloads on Microsoft ... of their technology stack complies with FDA and global regulations. USDM's new managed ...
(Date:7/4/2020)... ... July 03, 2020 , ... ... outstanding recognition and multiple awards for not only the products and treatments developed, ... Skincare and Vivace® Microneedle RF. All the brands built by ABM have received ...
(Date:6/28/2020)... ... June 25, 2020 , ... ... software solutions for biopharmaceutical R&D, today announced that it has entered into ... full-length multispecific antibodies (Multiclonics®), to support their translational and clinical research strategy ...
Breaking Biology News(10 mins):
(Date:7/31/2020)... SHREWSBURY, Mass. (PRWEB) , ... July 29, 2020 ... ... clad wire that can combine up to three different materials for use with ... can combine up to three materials to meet specific design requirements, performance, and ...
(Date:7/22/2020)... ... July 22, 2020 , ... Join experts from ... Sr. Manager Regulatory Solutions, in a one hour live webinar on Thursday, ... body in China for drugs and medical devices. Specifically, for medical devices, the NMPA ...
(Date:7/10/2020)... Va. (PRWEB) , ... July 08, 2020 , ... ... testing services and products, announces a significant expansion of laboratory operations through its ... collection for agencies implementing testing programs. , Bode-CARES provides a ...
(Date:7/7/2020)... BOSTON (PRWEB) , ... July 06, 2020 , ... ... Innovative Practices Awards. Entries from Roche, Eli Lilly, Bristol-Myers Squibb, the University of ... Since 2003, Bio-IT World has hosted an elite awards program, highlighting outstanding examples ...
Breaking Biology Technology:
Cached News: