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“To develop a screening test for the detection of disease-specific markers to prevent the transmission of vCJD (the human variant of ‘Mad Cow disease’) via blood donation.”
 

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.

Prionscreen  Screening donated blood to prevent CJD transmission

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

 
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