Navigation Links
Mutant proteins result in infectious prion disease in mice
Date:12/5/2008

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."


'/>"/>

Contact: Debra Kain
ddkain@ucsd.edu
619-543-6163
University of California - San Diego
Source:Eurekalert

Related medicine news :

1. What is the more suitable for early detection of low abundant lamivudine-resistant mutants?
2. FSU researchers mutant proteins could lead to new treatment for heart disease
3. Mutant gene causes epilepsy, intellectual disability in women
4. Effect of mutant p53 stability on tumorigenesis and drug design
5. Intrabody can mop up mutant protein in Huntingtons disease model
6. Mutant Mouse Gene Offers Clues to Hearing Loss
7. Penn study finds pro-death proteins required to regulate healthy immune function
8. Heat shock proteins are co-opted for cancer
9. Loss of gene leads to protein splicing and buildup of toxic proteins in neurons
10. Immune Proteins Warn of Early Lung Cancer
11. 2 proteins may be survival markers in some breast cancers
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:5/24/2017)... ... May 24, 2017 , ... Technique, technique, technique – with a ... lifting weights for strength training and exercise or simply lifting heavy objects, advises Dr. ... position is everything,” Dr. Chang says. “Improper technique in lifting anything heavy or an ...
(Date:5/23/2017)... (PRWEB) , ... May 23, 2017 , ... ... Population Health and Clinical Integration company, announced today that its iClinic V12.2 solution ... (PCMH) 2017 Prevalidation. NCQA recently introduced PCMH 2017 standards which emphasize team-based ...
(Date:5/23/2017)... PITTSBURGH, Pa. (PRWEB) , ... May 23, 2017 , ... ... today plans to open The Alexis Joy D’Achille Center for Women’s Behavioral Health at ... state-of-the-art care available in western Pennsylvania for women suffering from pregnancy-related depression. Construction ...
(Date:5/23/2017)... ... May 23, 2017 , ... Cambridge, MA, May 23, 2017—Total ... according to a Workers Compensation Research Institute (WCRI) study, a contrast from the ... System: CompScope™ Benchmarks, 17th Edition looks at indemnity and medical payments for ...
(Date:5/23/2017)... ... May 23, 2017 , ... i2i Population Health, ... in KLAS category winner, has named Daniel P. Bullington as chief technology officer. ... technology platform and product offerings,” says Justin Neece, president. “Daniel is an excellent ...
Breaking Medicine News(10 mins):
(Date:5/18/2017)... , May 18, 2017  Two Bayer U.S. ... Association (HBA) during its recent 28 th ... City.  The event showcases HBA,s longstanding mission of furthering ... of healthcare. Cindy Powell-Steffen , senior ... U.S. Radiology division, and Libby Howe , a ...
(Date:5/11/2017)... , May 11, 2017  Thornhill Research ... has been awarded an $8,049,024 USD five-year, firm-fixed-priced, ... through the Canadian Commercial Corporation (CCC) ( ... Anesthesia Systems to administer general anesthesia to patients ... "The US Marine Corps have been ...
(Date:5/10/2017)... BURNIE, Md. , May 10, 2017 ... retention solutions for the clinical research industry, is proud ... www.CSSiEnroll.com . The new website features both enriched ... overall user experience and enhances the company,s already well-established ... the industry. "After many months of ...
Breaking Medicine Technology: