Featured Research

from universities, journals, and other organizations

Study Sheds Light On Age-related Changes Of Prion Diseases

Date:
April 27, 2005
Source:
Case Western Reserve University
Summary:
In a laboratory study, a mutant prion protein was found to assume its normal shape in the cell, but after it was unfolded it failed to return to its normal shape. This may explain why a prion disease called GSS is age-related since the healthy young cell can properly fold and maintain the mutant protein, but deficits in cellular machinery associated with aging might promote accumulation of the aberrant form of the prion protein resulting in disease.

A new study sheds light on why normal prion proteins may experience age-related mutations in inherited diseases. Researchers at the Case Western Reserve University School of Medicine studied a previously discovered mutation in the prion protein in members of an extended family in Indiana who inherited the prion disease Gerstmann-Straussler-Sheinker (GSS). GSS is a familial disease that causes movement disorders and has a course of approximately 3 years. This disease, and other prion diseases such as mad cow disease, are believed to be caused by the mutation of normally occurring proteins called prion proteins and are inevitably fatal.

Related Articles


In a laboratory study of the mutation that causes GSS in this family the mutant protein was found to assume its normal shape in the cell, but after it was unfolded it failed to return to its normal shape. This may explain why the disease is age-related since the healthy young cell can properly fold and maintain the mutant protein, but the deficits in the cellular machinery associated with aging might promote accumulation of the aberrant form of the prion protein resulting in disease.

There are several remaining questions, according to Robert Petersen, Ph.D., associate professor of pathology and neuroscience at Case and senior author of the study. "The most important question is," he said, "what is the exact nature of the improperly refolded form of the mutant protein. In addition, it will be important to address what features of the aging cell are most involved in defective protein folding, the understanding of which may result in novel therapeutic strategies for halting these devastating diseases."

###

The study appears in the April 2005 issue of the Journal of Alzheimer's Disease (http://www.j-alz.com), published by IOS Press.


Story Source:

The above story is based on materials provided by Case Western Reserve University. Note: Materials may be edited for content and length.


Cite This Page:

Case Western Reserve University. "Study Sheds Light On Age-related Changes Of Prion Diseases." ScienceDaily. ScienceDaily, 27 April 2005. <www.sciencedaily.com/releases/2005/04/050426215458.htm>.
Case Western Reserve University. (2005, April 27). Study Sheds Light On Age-related Changes Of Prion Diseases. ScienceDaily. Retrieved March 4, 2015 from www.sciencedaily.com/releases/2005/04/050426215458.htm
Case Western Reserve University. "Study Sheds Light On Age-related Changes Of Prion Diseases." ScienceDaily. www.sciencedaily.com/releases/2005/04/050426215458.htm (accessed March 4, 2015).

Share This


More From ScienceDaily



More Mind & Brain News

Wednesday, March 4, 2015

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

This Nasal Treatment Could Help Ease Migraine Pain

This Nasal Treatment Could Help Ease Migraine Pain

Newsy (Mar. 2, 2015) Researchers gave lidocaine to 112 patients, and about 88 percent of the subjects said they needed less migraine-relief medicine the next day. Video provided by Newsy
Powered by NewsLook.com
How Facebook Use Can Lead To Depression

How Facebook Use Can Lead To Depression

Newsy (Mar. 1, 2015) Margaret Duffy of the University of Missouri talks about her study on the social network and the envy and depression that Facebook use can cause. Video provided by Newsy
Powered by NewsLook.com
The Best Foods to Battle Stress

The Best Foods to Battle Stress

Buzz60 (Feb. 26, 2015) If you&apos;re dealing with anxiety, there are a few foods that can help. Krystin Goodwin (@krystingoodwin) has the best foods to tame stress. Video provided by Buzz60
Powered by NewsLook.com
Sleeping Too Much Or Too Little Might Increase Stroke Risk

Sleeping Too Much Or Too Little Might Increase Stroke Risk

Newsy (Feb. 26, 2015) People who sleep more than eight hours per night are 45 percent more likely to have a stroke, according to a University of Cambridge study. 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:

Strange & Offbeat Stories


Health & Medicine

Mind & Brain

Living & Well

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