News Release

New insights into the diversity of Creutzfeldt-Jakob disease agents

Peer-Reviewed Publication

PLOS

Researchers from the United Kingdom and France have identified four separate biochemical subgroups in a selection of cases of Creutzfeldt-Jakob disease. The study, published March 14th in the open-access journal PLoS Pathogens, suggests that these subgroups could represent distinct prion strains in what is the most common human prion disease.

Prion diseases are transmissible neurodegenerative disorders characterized by accumulation of an abnormal isoform (PrPSc) of a host-encoded protein (PrPC) in affected tissues. Although considered a spontaneous disorder, the clinicopathological characteristics of sporadic Creutzfeldt-Jakob disease (sCJD) are variable and substantially influenced by a particular variation in the DNA of the prion protein gene (PRNP). Due to the strong influence of host factors on the characteristics of the disease, diversity of prion agents responsible for CJD remains extremely difficult to investigate.

In this study, using two new biochemical assays, the authors identified four distinct biochemical PrPSc subgroups in 41 sCJD cases. These subgroups correlate with the current sCJD subclassification. The subgroups were also found in 12 iatrogenic CJD (iCJD) cases from different countries. Iatrogenic CJD occurs following human-to-human sCJD transmission. In contrast to the sCJD cases, however, there was no particular correlation with the PRNP codon in the iCJD cases, indicating that observed biochemical properties could be specific to the prion agent.

Further studies are required to confirm that the four biochemical subgroups identified correlate with distinct biological infectious agents.

###

PLEASE ADD THIS LINK TO THE PUBLISHED ARTICLE IN ONLINE VERSIONS OF YOUR REPORT: http://www.plospathogens.org/doi/ppat.1000029

CITATION: Uro-Coste E, Cassard H, Simon S, Lugan S, Bilheude J-M, et al. (2008) Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease. PLoS Pathog 4(3): e1000029. doi:10.1371/journal.ppat.1000029

CONTACT:
Dr. Olivier Andreoletti
UMR INRA-ENVT
Toulouse, France
+33-561-193895
o.andreoletti@envt.fr

Disclaimer

This press release refers to an upcoming article in PLoS Pathogens. The release is provided by the article authors and/or their institutions. Any opinions expressed in these releases or articles are the personal views of the journal staff and/or article contributors, and do not necessarily represent the views or policies of PLoS. PLoS expressly disclaims any and all warranties and liability in connection with the information found in the releases and articles and your use of such information.

About PLoS Pathogens

PLoS Pathogens (www.plospathogens.org) publishes outstanding original articles that significantly advance the understanding of pathogens and how they interact with their host organisms. All works published in PLoS Pathogens are open access. Everything is immediately available subject only to the condition that the original authorship and source are properly attributed. Copyright is retained by the authors. The Public Library of Science uses the Creative Commons Attribution License.

About the Public Library of Science

The Public Library of Science (PLoS) is a non-profit organization of scientists and physicians committed to making the world's scientific and medical literature a freely available public resource. For more information, visit http://www.plos.org.


Disclaimer: AAAS and EurekAlert! are not responsible for the accuracy of news releases posted to EurekAlert! by contributing institutions or for the use of any information through the EurekAlert system.