Featured Research

from universities, journals, and other organizations

The shape of infectious prions

Date:
January 24, 2014
Source:
Sissa Medialab
Summary:
Prions are unique infective agents -- unlike viruses, bacteria, fungi and other parasites, prions do not contain either DNA or RNA. Despite their seemingly simple structure, they can propagate their pathological effects like wildfire, by "infecting" normal proteins. PrPSc (the pathological form of the prion protein) can induce normal prion proteins (PrPC) to acquire the wrong conformation and convert into further disease-causing agents.

Structural changes were located in the prion protein N-terminus, where a novel reorganization of the beta sheet (in yellow) was observed. In the background, the X-ray diffraction pattern of the crystal composed by the complex prion protein-Nanoboy.
Credit: SISSA

Prions are unique infective agents -- unlike viruses, bacteria, fungi and other parasites, prions do not contain either DNA or RNA. Despite their seemingly simple structure, they can propagate their pathological effects like wildfire, by "infecting" normal proteins. PrPSc (the pathological form of the prion protein) can induce normal prion proteins (PrPC) to acquire the wrong conformation and convert into further disease-causing agents.

Related Articles


"When they are healthy, they look like tiny spheres; when they are malignant, they appear as cubes" stated Giuseppe Legname, principal investigator of the Prion Biology Laboratory at the Scuola Internazionale Superiore di Studi Avanzati (SISSA) in Trieste, when describing prion proteins. Prions are "misfolded" proteins that cause a group of incurable neurodegenerative diseases, including spongiform encephalopathies (for example, mad cow diseases) and Creutzfeldt-Jakob disease. Legname and coworkers have recently published a detailed analysis of the early mechanisms of misfolding. Their research has just been published in the Journal of the American Chemical Society, the most authoritative scientific journal in the field.

"For the first time, our experimental study has investigated the structural elements leading to the disease-causing conversion" explains Legname. "With the help of X-rays, we observed some synthetic prion proteins engineered in our lab by applying a new approach -- we used nanobodies, i.e. small proteins that act as a scaffolding and induce prions to stabilize their structure." Legname and colleagues reported that misfolding originates in a specific part of the protein named "N-terminal." "The prion protein consists of two subunits. The C-terminal has a clearly defined and well-known structure, whereas the unstructured N-terminal is disordered, and still largely unknown. This is the very area where the early prion pathological misfolding occurs" adds Legname. "The looser conformation of the N-terminal likely determines a dynamic structure, which can thus change the protein shape."

"Works like ours are the first, important steps to understand the mechanisms underlying the pathogenic effect of prions" concludes Legname. "Elucidating the misfolding process is essential to the future development of drugs and therapeutic strategies against incurable neurodegenerative diseases."


Story Source:

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


Journal Reference:

  1. Romany N. N. Abskharon, Gabriele Giachin, Alexandre Wohlkonig, Sameh H. Soror, Els Pardon, Giuseppe Legname, Jan Steyaert. Probing the N-Terminal β-Sheet Conversion in the Crystal Structure of the Human Prion Protein Bound to a Nanobody. Journal of the American Chemical Society, 2014; 136 (3): 937 DOI: 10.1021/ja407527p

Cite This Page:

Sissa Medialab. "The shape of infectious prions." ScienceDaily. ScienceDaily, 24 January 2014. <www.sciencedaily.com/releases/2014/01/140124082602.htm>.
Sissa Medialab. (2014, January 24). The shape of infectious prions. ScienceDaily. Retrieved November 24, 2014 from www.sciencedaily.com/releases/2014/01/140124082602.htm
Sissa Medialab. "The shape of infectious prions." ScienceDaily. www.sciencedaily.com/releases/2014/01/140124082602.htm (accessed November 24, 2014).

Share This


More From ScienceDaily



More Plants & Animals News

Monday, November 24, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Stray Dog Follows Adventure Racing Team for 6-Day Endurance Race

Stray Dog Follows Adventure Racing Team for 6-Day Endurance Race

Buzz60 (Nov. 24, 2014) A Swedish Adventure racing team travels to try and win a world title, but comes home with something way better: a stray dog that joined the team for much of the grueling 430-mile race. Jen Markham has the story. Video provided by Buzz60
Powered by NewsLook.com
Deadly Japanese Pufferfish Discovered in Crimean Waters

Deadly Japanese Pufferfish Discovered in Crimean Waters

Reuters - Light News Video Online (Nov. 24, 2014) The capture of deadly Japanese pufferfish in the waters of Crimea is causing concern for fishermen and scientists alike. Sharon Reich reports. Video provided by Reuters
Powered by NewsLook.com
Terrifying Black Seadevil Fish Captured on First-of-Its Kind Video

Terrifying Black Seadevil Fish Captured on First-of-Its Kind Video

Buzz60 (Nov. 24, 2014) An aquarium captures a first-of-its kind video of a notoriously camera-shy fish that’s also not so camera-friendly. Jen Markham explains. Video provided by Buzz60
Powered by NewsLook.com
Red Panda Cubs Explore the Bratislava Zoo

Red Panda Cubs Explore the Bratislava Zoo

AFP (Nov. 24, 2014) Four-month old Red Panda twins Pim and Pam still rely on their mother for breast milk at the Bratislava Zoo in Slovakia, but the precocious cubs have begun to branch out to solid foods, as well. Duration: 00:41 Video provided by AFP
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


Plants & Animals

Earth & Climate

Fossils & Ruins

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