Objective
To monitor the Europe-wide incidence of neuropathologically confirmed human transmissible spongiform encephalopathies (TSEs, prion diseases) both prospectively and retrospectively.
To monitor the clinicopathological pattern of human TSEs in Europe and possible emergence of new patterns.
To characterise the range of the phenotypic spectrum of human TSEs, with emphasis on detailed neuropathological assessment. In particular, the new variant of CJD will be searched both prospectively and retrospectively on a Europe-wide scale. Moreover, this neuropathological assessment might serve as basis for a redefinition of criteria for diagnosis and classification of human TSEs.
To further characterise the link between human TSE phenotypes and the genotype of the prion protein (PrP) gene (PRNP) by assessing the distribution of PRNP mutations and relevant polymorphisms in Europe.
To clarify the pathogenesis of tissue damage in human TSEs, especially the relation of PrP deposition and tissue lesioning.
To further characterise other neurodegenerative conditions suspected as prion diseases such as progressive subcortical gliosis.
To develop improved diagnostic tools, including antibodies which might be able to discriminate between the normal and the pathological isoforms of PrP, and a standard for laboratory diagnostics of TSEs.
TSEs represent a unique paradigm in biomedicine: they can be both infectious and hereditary, with the intriguing "prion" hypothesis of infectivity conferred by a proteinase resistant (prion) protein (PrP) only. Moreover, world-wide concern has been emerging about a link between human diseases and those in animals, most notably bovine spongiform encephalopathy (BSE). For a most recently recognised new variant of Creutzfeldt-Jakob disease (nv-CJD) in the UK and France, origin from BSE has become probable. Human TSEs can be definitely diagnosed only by neuropathological examination. For diagnosis of nv-CJD, autopsy confirmation is mandatory. This project aims to gain further insight into the various phenotypes of human TSEs, their distribution and pathogenesis. Coordination of its tasks will exploit the infrastructure of a European network of laboratories involved in diagnosis of, and basic research in, human TSEs. The European database of human TSEs, the Tissue Bank, and the Clearing House for atypical cases will be expanded and supplemented by additional research into PRNP genotypes and pathogenesis of CNS tissue lesions, and by development of improved diagnostic tools such as new antibodies.
Fields of science (EuroSciVoc)
CORDIS classifies projects with EuroSciVoc, a multilingual taxonomy of fields of science, through a semi-automatic process based on NLP techniques. See: The European Science Vocabulary.
CORDIS classifies projects with EuroSciVoc, a multilingual taxonomy of fields of science, through a semi-automatic process based on NLP techniques. See: The European Science Vocabulary.
- natural sciences computer and information sciences databases
- natural sciences biological sciences biochemistry biomolecules proteins
- natural sciences biological sciences genetics mutation
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Programme(s)
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Multi-annual funding programmes that define the EU’s priorities for research and innovation.
Topic(s)
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Calls for proposals are divided into topics. A topic defines a specific subject or area for which applicants can submit proposals. The description of a topic comprises its specific scope and the expected impact of the funded project.
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Funding Scheme
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Funding scheme (or “Type of Action”) inside a programme with common features. It specifies: the scope of what is funded; the reimbursement rate; specific evaluation criteria to qualify for funding; and the use of simplified forms of costs like lump sums.
Coordinator
1097 WIEN
Austria
The total costs incurred by this organisation to participate in the project, including direct and indirect costs. This amount is a subset of the overall project budget.