Featured Research

from universities, journals, and other organizations

Most lethal known species of prion protein identified

Date:
February 9, 2012
Source:
Scripps Research Institute
Summary:
Scientists have identified a single prion protein that causes neuronal death similar to that seen in "mad cow" disease, but is at least 10 times more lethal than larger prion species.

Scientists from the Florida campus of The Scripps Research Institute have identified a single prion protein that causes neuronal death similar to that seen in “mad cow” disease, but is at least 10 times more lethal than larger prion species.

This toxic single molecule or “monomer” challenges the prevailing concept that neuronal damage is linked to the toxicity of prion protein aggregates called “oligomers.”

The study was published this week in an advance, online edition of the journal Proceedings of the National Academy of Sciences.

“By identifying a single molecule as the most toxic species of prion proteins, we’ve opened a new chapter in understanding how prion-induced neurodegeneration occurs,” said Scripps Florida Professor Corinne Lasmézas, who led the new study.  “We didn’t think we would find neuronal death from this toxic monomer so close to what normally happens in the disease state. Now we have a powerful tool to explore the mechanisms of neurodegeneration.”

In the study, the newly identified toxic form of abnormal prion protein, known as TPrP, caused several forms of neuronal damage ranging from apoptosis (programmed cell death) to autophagy, the self-eating of cellular components, as well as molecular signatures remarkably similar to that observed in the brains of prion-infected animals. The study found the most toxic form of prion protein was a specific structure known as alpha-helical.

New Paths to Explore

In addition to the insights it offers into prion diseases such as “mad cow” and a rare human form Creutzfeldt-Jakob disease, the study opens the possibility that similar neurotoxic proteins might be involved in neurodegenerative disorders such as Alzheimer’s and Parkinson diseases.

In prion disease, infectious prions (short for proteinaceous infectious particles), thought to be composed solely of protein, have the ability to reproduce, despite the fact that they lack DNA and RNA. Mammalian cells normally produce what is known as cellular prion protein or PrP; during infection with a prion disease, the abnormal or misfolded protein converts the normal host prion protein into its disease form.

Lasmézas explains that prion diseases are similar to Alzheimer's and other protein misfolding diseases in that they are caused by the toxicity of a misfolded host protein. Recent work, as reported in The New York Times, also found that diseases such as Alzheimer's resemble prion diseases by spreading from cell to cell.

The new study adds another twist. “Until now, it was thought that oligomers of proteins are toxic in all these diseases,” Lasmézas said. “Since we found for the first time that an abnormally folded monomer is highly toxic, it opens up the possibility that this might be true also for some other protein misfolding diseases as well.”


Story Source:

The above story is based on materials provided by Scripps Research Institute. Note: Materials may be edited for content and length.


Journal Reference:

  1. M. Zhou, G. Ottenberg, G. F. Sferrazza, C. Ida Lasmezas. Highly neurotoxic monomeric  -helical prion protein. Proceedings of the National Academy of Sciences, 2012; DOI: 10.1073/pnas.1118090109

Cite This Page:

Scripps Research Institute. "Most lethal known species of prion protein identified." ScienceDaily. ScienceDaily, 9 February 2012. <www.sciencedaily.com/releases/2012/02/120209152814.htm>.
Scripps Research Institute. (2012, February 9). Most lethal known species of prion protein identified. ScienceDaily. Retrieved April 23, 2014 from www.sciencedaily.com/releases/2012/02/120209152814.htm
Scripps Research Institute. "Most lethal known species of prion protein identified." ScienceDaily. www.sciencedaily.com/releases/2012/02/120209152814.htm (accessed April 23, 2014).

Share This



More Plants & Animals News

Wednesday, April 23, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Raw: Leopard Bites Man in India

Raw: Leopard Bites Man in India

AP (Apr. 22, 2014) — A leopard caused panic in the city of Chandrapur on Monday when it sprung from the roof of a house and charged at rescue workers. (April 22) Video provided by AP
Powered by NewsLook.com
Iowa College Finds Beauty in Bulldogs

Iowa College Finds Beauty in Bulldogs

AP (Apr. 22, 2014) — Drake University hosts 35th annual Beautiful Bulldog Contest. (April 21) Video provided by AP
Powered by NewsLook.com
805-Pound Shark Caught Off The Coast Of Florida

805-Pound Shark Caught Off The Coast Of Florida

Newsy (Apr. 22, 2014) — One Florida fisherman caught a 805-pound shark off the coast of Florida earlier this month. Video provided by Newsy
Powered by NewsLook.com
Breakfast Foods Are Getting Pricier

Breakfast Foods Are Getting Pricier

AP (Apr. 21, 2014) — Breakfast is now being served with a side of sticker shock. The cost of morning staples like bacon, coffee and orange juice is on the rise because of global supply problems. (April 21) Video provided by AP
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