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:2/9/2016)... (NASDAQ: AWRE ), a leading supplier of biometrics software and ... ended December 31, 2015.  --> ... million, an increase of 61% compared to $4.3 million in the ... 2015 was $2.6 million compared to $0.2 million in the fourth ... Higher revenue and operating income in the fourth quarter of ...
(Date:2/4/2016)... 2016 The field of Human Microbiome ... most popular hubs of the biotechnology industry. While ... studies of human microbiota, have garnered a lot ... microbiome space has literally exploded in terms of ... report focuses on biomedical aspects of research, development, ...
(Date:2/3/2016)... http://www.researchandmarkets.com/research/d8zjcd/emotion_detection ) ... "Emotion Detection and Recognition Market by Technology ... Tools (Facial Expression, Voice Recognition and Others), ... Global forecast to 2020" report to ... ) has announced the addition of the ...
Breaking Biology News(10 mins):
(Date:2/11/2016)... 2016 Non-profit Consortium Aims to Generate ... Support Research and Discovery --> ... ambitious plan to sequence 100,000 individuals. It is intended to ... least 7 of North and East Asian countries. ... the project will focus on creating phased reference genomes for ...
(Date:2/11/2016)... and GERMANTOWN, Maryland , February 11, ... Standard: QIA) today announced the introduction of more than ... profiling, expanding QIAGEN,s portfolio of Sample to Insight solutions ... select from over 20,000 human genes and lncRNA to ... cellular phenotypes and disease processes. --> QGEN ...
(Date:2/11/2016)... -- Wellcentive today announced it has been selected by FamilyCare ... community care organization (CCO) with more than 130,000 ... and care management solutions and services. Wellcentive,s capabilities ... managers, analysts and care managers while providing insight ... members. Oregon . ...
(Date:2/11/2016)... DELRAY BEACH, Florida , February 11, 2016 ... --> PositiveID Corporation ("PositiveID" or "Company") (OTCQB: ... and diagnostics, announced today that its Thermomedics subsidiary, ... significant progress on its growth plan in January ... healthcare products distributors, increasing sequential monthly sales growth, ...
Breaking Biology Technology: