The problem

Transmissible spongiform encephalopathy (TSE) is associated with abnormal prion proteins in the brain. Research has confirmed that the human variant of Creutzfeldt-Jakob disease (vCJD) – more familiarly known as ‘Mad Cow disease’ – is transmissible through blood donation. The use of blood and blood products from sub-clinically infected donors creates a significant risk for secondary transmission and the persistence of vCJD in the population. Post-mortem brain tissue is currently used to diagnose vCJD. An assay is urgently required to screen blood donors for abnormal prions at an early stage of infection.

Overview:

Prionscreen aims to develop a screening assay to detect prion proteins in blood.

The project will:

• Identify new biochemical markers for CJD and other prion diseases using state-of-the-art techniques, including proteomics and genomic analysis
• Establish sample and data banks for infected blood and brain tissue
• Test potential biomarkers using infected tissue samples
• Develop microfiltration assays for the detection of prions in the blood of experimentally infected animals and from human tissue samples collected at an early disease stage
• Assess the sensitivity and specificity of the methodology in comparison to other methods.

Contribution to policy development:

• A routine screening assay for the preclinical diagnosis of vCJD will lead to improved estimates of the number of infected individuals in Europe.
• More accurate figures for disease incidence will have relevance for public health policy.
• Enhanced knowledge of disease mechanisms and transmission will inform potential therapeutic or prevention strategies.

Project deliverables

• A bank for serum, plasma and other biological samples from patients with a variety of neurogenerative diseases – March 2008
• Characterisation of markers that interact with prion protein aggregates, and a microfiltration assay for detection of prions in experimentally infected animals – September 2008
• A microfiltration assay for the detection of prion protein aggregates in human blood – September 2009
• Database of biomarkers and protein sequences for the detection of human spongiform encephalopathies – March 2010
• Tested procedures for the early diagnosis of vCJD – March 2010

Dissemination

• Project meetings – June and December 2007, June 2008, March 2009, November 2009
• Web-based platform for information exchange – March 2008
• Newsletters – September 2007, 2008, 2009
• Interim and final report – September 2008, 2010
• Presentations at conferences and publications in peer-reviewed scientific journals – March 2010

Technical information

Project acronym: Prionscreen
Project’s official full title: Development of a blood screening assay for diagnosis of prion diseases in humans
Research priority: Public health issues, including epidemiology contributing to disease prevention and responses to emerging rare and communicable diseases, allergies, procedures for secure blood and organ donations, non-animal test methods
Specific webpage: Not yet available
Proposal/contract no: 044438
Start date: March 2007
Kick off meeting: March 2007
Completion date: February 2010
European Commission scientific officer: Cornelius Schmaltz, Cornelius.SCHMALTZ@ec.europa.eu

Coordinator:
Name: Bereich Humanmedizin der Georg-August-Universität Göttingen
Abbreviated name: UKG-GOE
Address: National TSE Reference Centre
Dept. of Neurology
Georg-August University
37075 Göttingen
Country: Germany
Tel: +49 (0)551-396636
Fax: +49 (0)551-397020
Website: http://www.neurologie.med.uni-goettingen.de/, http://www.cjd-goettingen.de/

Partners

Name: Robert Koch-Institut
Abbreviated name: RKI
Country: Germany

Name: Istituto Superiore di Sanità
Abbreviated name: ISS
Country: Italy

Name: Erasmus University Rotterdam
Abbreviated name: EUR
Country: Netherlands

Name: Slovak Medical University – Research Base
Abbreviated name: SMU RB
Country: Slovakia

Name: National CJD Surveillance Unit Edinburgh
Abbreviated name: UEDIN
Country: United Kingdom