Navigation Links
The origin of the evil conformation
Date:1/24/2014

"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.

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.

"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".


'/>"/>

Contact: Federica Sgorbissa
federica@medialab.sissa.it
0039-040-378-7644
International School of Advanced Studies (SISSA)
Source:Eurekalert  

Related biology news :

1. A 21st century adaptation of the Miller-Urey origin of life experiments
2. Spinal cord findings could help explain origins of limb control
3. The origin of flowers: DNA of storied plant provides insight into the evolution of flowering plants
4. Reinterpreting origins, examining skeletal preservation, and understanding volcanics
5. Fossils clarify the origins of wasps and their kin: alderfly ancestors, snakefly cousins
6. Origin of species: Protein imbalances doom hybrids
7. Researchers uncover origins of cattle farming in China
8. U of M scientists solve major piece in the origin of biological complexity
9. Genetic study proves Israels wild boars originated in Europe
10. Bats confirmed as SARS origin
11. Did brain tumor stem cells originate from malignant neural stem cells?
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
The origin of the evil conformation
(Date:2/7/2017)... , February 7, 2017 ... Global Solutions Corporation [OTC: IDGS], ("Ipsidy" or the "Company") ... electronic transaction processing services, is pleased to announce the ... Company. Effective January 31, 2017, Philip ... of Directors, CEO and President.  An experienced payment industry ...
(Date:2/3/2017)...  Texas Biomedical Research Institute announced that its Board of ... as the Institute,s new President and CEO. Dr. Schlesinger will ... He is currently the Chair of the Department of Microbial ... Interface Biology at Ohio State University. "We are ... CEO of Texas Biomed," said Dr. James O. Rubin ...
(Date:2/2/2017)... Feb. 2, 2017   TapImmune, Inc. ... company specializing in the development of innovative peptide ... of cancer and metastatic disease, announced today it ... manufacturing of a second clinical lot of TPIV ... receptor alpha. The manufactured vaccine product will be ...
Breaking Biology News(10 mins):
(Date:2/23/2017)... JOSE, Calif. , Feb. 23, 2017 /PRNewswire/ ... exclusive license for two key immunotherapy technologies from ... first technology provides a method to monitor a ... such as PD-L1 and CTLA-4.  The second license ... if a patient is likely to have an ...
(Date:2/23/2017)... San Antonio, TX (PRWEB) , ... February 23, ... ... and Drug Administration (FDA) de novo clearance to begin marketing the SPEAC® System, ... is indicated for adults at home or in healthcare facilities during periods of ...
(Date:2/23/2017)... ... ... The Greater Gift Initiative, Inc , (GGI) a Winston-Salem, NC 501(c)3 non-profit, ... is to advance global health and highlight the greater good of clinical trial participation ... trial volunteer. The vision of GGI is to serve as a philanthropic connector between ...
(Date:2/23/2017)... ... February 22, 2017 , ... Seventy-one ... been named Fellows of the Society this year, the Fellows Committee has announced. ... fields of optics, photonics, and imaging as well as their service to the ...
Breaking Biology Technology: