HOME >> BIOLOGY >> NEWS
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
kristac@stanford.edu
Stanford University Medical Center
30-Jan-2003


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:
*Name:
*Comment:
*Email:


(Date:4/1/2020)... ... April 01, 2020 , ... The ... named Jean-François Duliere as a European Regulatory Advisor. Effective 1 April 2020, Duliere ... and facilities design for biotechnology, oral solid dosage (OSD), and sterile manufacturing. , ...
(Date:3/20/2020)... ... March 20, 2020 , ... ImStem Biotechnology, Inc. ... (ESC) derived mesenchymal stem cells (hES-MSC), through a proprietary method using a trophoblast ... and rare orphan diseases, today announced that the U.S. Food and Drug Administration ...
(Date:3/13/2020)... ... 13, 2020 , ... According to the American Academy of ... possibly be transmitted by aerosol or direct contact with the conjunctiva, the superficial ... precautions to avoid contracting the coronavirus. Touching the eyes while inserting contact lenses ...
Breaking Biology News(10 mins):
(Date:3/10/2020)... ... March 10, 2020 , ... Ortho Clinical Diagnostics, a ... laboratory solutions, announced its ORTHOTM Sera, ID-MTS, a suite of extended antigen in a ... cleared to run on ORTHO VISION® and ORTHO VISION® Max in the United States ...
(Date:3/3/2020)... Ill. (PRWEB) , ... March 03, 2020 , ... ... company serving the pharmaceutical, biotech and medical device industries, was recognized by INC. ... 5,000 puts forward annual lists to note industry leaders greatly impacting their spheres ...
(Date:2/21/2020)... ... February 20, 2020 , ... In a free ... speakers from Synteract including Lisa Dilworth, BS, MS, Vice President Rare, Orphan ... challenges, with feasible strategies and solutions to support patients, sites and sponsors. , When ...
(Date:2/19/2020)... ... February 18, 2020 , ... Shoreline Biome, ... to the strain level, recently unveiled a novel DNA isolation product. , Shoreline ... isolation of high molecular weight (HMW) single-stranded DNA (ssDNA) in excess of 40 ...
Breaking Biology Technology:
Cached News: