Featured Research

from universities, journals, and other organizations

New Mouse Model Of Prion Disease: Mutant Proteins Result In Infectious Prion Disease In Mice

Date:
December 6, 2008
Source:
University of California - San Diego
Summary:
Scientists have created an infectious prion disease in a mouse model, in a step that may help unravel the mystery of this progressive disease that affects the nervous system in humans and animals.

A worldwide group of scientists has created an infectious prion disease in a mouse model, in a step that may help unravel the mystery of this progressive disease that affects the nervous system in humans and animals. The research team, including Christina J. Sigurdson, D.V.M., Ph.D., assistant professor of pathology at the University of California, San Diego School of Medicine, also discovered that changing the structure of the prion protein by altering just two nucleic acids leads to a fatal neurological disorder in mice.

Their findings will be published on line in Proceedings of the National Academy of Sciences (PNAS) the week of December 1.

The study, led by Professor Dr. Adriano Aguzzi of the Institute of Neuropathology at the University of Zurich in Switzerland, was designed to investigate the specific changes in the prion protein that may contribute to chronic wasting disease (CWD). CWD is a highly infectious prion disease found in free-ranging deer and elk that is similar to bovine spongiform encephalopathy (BSE, or "mad cow disease") in cattle and Creutzfeldt-Jakob disease in humans. Prion diseases are thought to be a result of a misfolded form of the prion protein that induces formation of amyloid plaques in the brain – changes that are also seen in patients with Alzheimer's disease.

By altering two nucleic acids in the prion gene, the researchers developed a transgenic mouse model that expressed the mutant prion protein. These changes resulted in a "loop" in the protein structure of the mice that was rigid – similar to the structure of the elk prion protein, and unlike the flexible "loop" found in normal mouse or human prion proteins. Aging mice with the "rigid loop" prion protein accumulated plaques in the brain and developed symptoms of neurological disease that are features of prion-related disorders.

"It could be that this 'loop' region of the protein can promote the formation of amyloid plaques in the brain," said Sigurdson. "We also found that by transferring brain tissue from mice with the mutant protein into mice expressing the normal mouse prion protein, we could transmit the neurologic disease between the two groups of animals."

According to Sigurdson, the discovery that an infectious disease can be generated through just two mutations in the prion gene is of particular interest. "Some forms of prion disease in humans caused by genetic mutations have also been shown to be infectious," she said. "This new mouse model of the disease may be useful in our understanding of how the misfolded protein leads to neurodegeneration and for testing new therapies against prion disease."

Additional contributors to the study include K. Peter R. Nilsson, Mathias Heikenwälder, Giuseppe Manco, Petra Schwarz, David Ott, Christian Julius and Jeppe Falsig of the University of Zurich; Simone Hornemann, ETH Zürich; Thomas Rülicke, Austria University of Veterinary Medicine, Vienna; Pawel Liberski, Medical University Lodz, Poland; Lothar Stitz, Friedrich-Loeffler-Institute, Tübingen, Germany; and Kurt Wüthrich, The Scripps Research Institute, La Jolla, CA.

This study was supported by the European Union, the Swiss National Science Foundation, the National Competence Centers for Research on Neural Plasticity and Repair, and on Structural Biology, the National Institutes of Health, the Foundation for Research at the University of Zürich, the US National Prion Research Program, the Knut and Alice Wallenberg Foundation the Bonizzi-Theler Foundation, and by the ETH Zürich.


Story Source:

The above story is based on materials provided by University of California - San Diego. Note: Materials may be edited for content and length.


Cite This Page:

University of California - San Diego. "New Mouse Model Of Prion Disease: Mutant Proteins Result In Infectious Prion Disease In Mice." ScienceDaily. ScienceDaily, 6 December 2008. <www.sciencedaily.com/releases/2008/12/081205122936.htm>.
University of California - San Diego. (2008, December 6). New Mouse Model Of Prion Disease: Mutant Proteins Result In Infectious Prion Disease In Mice. ScienceDaily. Retrieved July 28, 2014 from www.sciencedaily.com/releases/2008/12/081205122936.htm
University of California - San Diego. "New Mouse Model Of Prion Disease: Mutant Proteins Result In Infectious Prion Disease In Mice." ScienceDaily. www.sciencedaily.com/releases/2008/12/081205122936.htm (accessed July 28, 2014).

Share This




More Plants & Animals News

Monday, July 28, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Traditional African Dishes Teach Healthy Eating

Traditional African Dishes Teach Healthy Eating

AP (July 28, 2014) — Classes are being offered nationwide to encourage African Americans to learn about cooking fresh foods based on traditional African cuisine. The program is trying to combat obesity, heart disease and other ailments often linked to diet. (July 28) Video provided by AP
Powered by NewsLook.com
Asteroid's Timing Was 'Colossal Bad Luck' For The Dinosaurs

Asteroid's Timing Was 'Colossal Bad Luck' For The Dinosaurs

Newsy (July 28, 2014) — The asteroid that killed the dinosaurs struck at the worst time for them. A new study says that if it hit earlier or later, they might've survived. Video provided by Newsy
Powered by NewsLook.com
Raw: Sea Turtle Hatchlings Emerge from Nest

Raw: Sea Turtle Hatchlings Emerge from Nest

AP (July 27, 2014) — A live-streaming webcam catches loggerhead sea turtle hatchlings emerging from a nest in the Florida Keys. (July 27) Video provided by AP
Powered by NewsLook.com
Russia Saves Gecko Sex Satellite, Media Has Some Fun With It

Russia Saves Gecko Sex Satellite, Media Has Some Fun With It

Newsy (July 27, 2014) — The satellite is back under ground control after a tense few days, but with a gecko sex experiment on board, the media just couldn't help themselves. Video provided by Newsy
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:
from the past week

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins