Objective
Neuronal ceroid-lipofuscinoses (NCLs) compose the most common type of inherited progressive encephalopathies in children. They are characterized by progressive death of cortical neurons and thus these rare diseases provide an excellent model to characterize molecular events resulting in neuronal degeneration. Using genomic approaches we have identified six out of seven proteins underlying NCLs: PPT1, CLN3, CLN5, CLN6, CLN8, and cathepsin D, the deficiencies of which result in neurodegenerative disorders with clinical features varying from an infantile lethal disease to relatively late onset neuropathological symptoms. Despite identification of these proteins the pathogenetic mechanisms of NCLs remain unclear. The underlying disease mechanisms must, however, be crucial to the survival of neurons. Consequently, this proposal is devoted to reveal the molecular pathways involved in NCL, the final goal being better understanding of the processes resulting in neuronal death. We will generate and use novel models for NCL: cell, yeast, nematode and mouse models at the cellular, tissue and whole organism level. Our first task will be to characterize the individual NCL proteins by studying their intracellular localization, transport and molecular interactions in neuronal cell models. The second task will be to generate and analyze yeast and nematode model organisms, which are extremely well suited for the dissection of the function of conserved NCL-proteins. The third task will be to analyze the pre-existing mouse models, mainly generated by the participants of this proposal, as well as to develop one novel mouse model for NCL. Special emphasis will be laid on the fourth task aiming to use advanced technologies of neuroanatomy and neurophysiology to address the mechanisms of neurodegeneration in these models. The fifth task is to develop bioinformatic tools and to expose putative novel metabolic pathways involved in NCL by gene express...
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 biological sciences neurobiology
- natural sciences biological sciences molecular biology molecular genetics
- natural sciences biological sciences biochemistry biomolecules proteins proteomics
- natural sciences biological sciences cell biology
- social sciences other social sciences development studies development economics
You need to log in or register to use this function
Keywords
Project’s keywords as indicated by the project coordinator. Not to be confused with the EuroSciVoc taxonomy (Fields of science)
Project’s keywords as indicated by the project coordinator. Not to be confused with the EuroSciVoc taxonomy (Fields of science)
Programme(s)
Multi-annual funding programmes that define the EU’s priorities for research and innovation.
Multi-annual funding programmes that define the EU’s priorities for research and innovation.
Topic(s)
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.
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.
Call for proposal
Procedure for inviting applicants to submit project proposals, with the aim of receiving EU funding.
Procedure for inviting applicants to submit project proposals, with the aim of receiving EU funding.
FP6-2002-LIFESCIHEALTH
See other projects for this call
Funding Scheme
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.
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
HELSINKI
Finland
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.