Objective
Mutations in certain proteins can cause their misfolding and aggregation, posing a potential threat to the health of an organism. Protein aggregation is counteracted by protein quality control (PQC) mechanisms that reduce the amounts of damaged molecules. As we age, however, the crucial surveillance mechanisms weaken and become less effective, leading to protein misfolding diseases. The most well-known protein disorders, or proteinopathies – Alzheimer's, Huntington's, and Parkinson's diseases – are characterized by the formation of toxic protein aggregates, known as amyloids. Current research focuses on such amyloid diseases, however, there seem to be many other, hidden protein disorders. In principle, every mutation in a protein can affect its folding and stability and may become pathological only when PQC mechanisms are compromised. To better understand this kind of proteinopathy, we will generate novel PQC tools and study muscle protein disorders in the nematode Caenorhabditis elegans. We will design dual-colour fluorescent protein reporters to monitor the misfolding and aggregation of disease-linked myosin variants, our model proteins. The ‘MyoState’ library, comprising hundreds of mutants with a broad range of folding defects, will allow us to study the pathological impact of individual myopathy mutations and investigate cellular rescue mechanisms. In C. elegans, myosin disease mutations cause characteristic defects in motility and muscle structure, which can be rescued by dietary restriction. This animal model thus provides us with a unique opportunity to perform genome-wide screens that will define the PQC network in muscle cells. By performing structural and functional analyses of key factors in this network, we aim to reveal the fundamental mechanisms of myosin quality control and its regulation. Our ultimate goal is to understand how the misfolding of functional proteins, whether due to mutation or age, impairs cell function.
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.
- medical and health sciences basic medicine neurology dementia alzheimer
- natural sciences biological sciences biochemistry biomolecules proteins
- natural sciences biological sciences genetics mutation
- medical and health sciences basic medicine neurology parkinson
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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.
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HORIZON.1.1 - European Research Council (ERC)
MAIN PROGRAMME
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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.
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.
HORIZON-ERC - HORIZON ERC Grants
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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.
(opens in new window) ERC-2023-ADG
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Net EU financial contribution. The sum of money that the participant receives, deducted by the EU contribution to its linked third party. It considers the distribution of the EU financial contribution between direct beneficiaries of the project and other types of participants, like third-party participants.
1030 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.