Objective
Mitochondria are the powerhouses of eukaryotic cells. Through oxidative phosphorylation (OxPhos), mitochondria extract energy from nutrients and use it to transfer protons across a proton-impermeable membrane creating a proton motive force to drive ATP production. Since the enzymes that catalyse OxPhos are encoded in both the mitochondrial and nuclear genomes, mutations in either one can cause mitochondrial disease, with a minimum prevalence of 1 in 5,000 live births in the EU. The molecular-biochemical causes of mitochondrial disorders and their clinical manifestations are highly diverse, but the most common defects are in complex I, the first enzyme of the OxPhos system. Currently, the mechanisms linking specific complex I mutations to OxPhos failure, cell and tissue damage, and ultimately disease, are poorly understood – precluding development of rational, evidence-based, therapies. The paucity of tools available to define mitochondrial function in the living cell is a major hindrance.
During my research in the United States, I have developed optical instrumentation, technology and expertise capable of defining the bioenergetic status of mitochondria in living cells, with an exquisite level of accuracy, under tightly defined conditions. Every bioenergetic parameter that impacts on OxPhos function is accessible, including the redox potential of both substrates of complex I, the proton motive force, and the flux. Here, I propose to transfer my unique instrumentation and expertise in cellular bioenergetics to the Mitochondrial Biology Unit in Cambridge, UK, and integrate them with existing projects on molecular and clinical aspects of mitochondrial disorders, in order to link the biochemical definition of complex I dysfunctions with the characterization of their consequences in vivo, provided by my technology. This integrated approach will allow us to bridge fundamental gaps in knowledge, and drive forward the development of effective clinical therapies.
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 physiology pathophysiology
- natural sciences biological sciences genetics mutation
- natural sciences biological sciences genetics genomes
- natural sciences biological sciences biochemistry biomolecules proteins enzymes
- natural sciences physical sciences optics spectroscopy
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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.
FP7-PEOPLE-2013-IIF
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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
W1B 1AL LONDON
United Kingdom
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.