Objective
Mammalian Target Of Rapamycin (mTOR) is an evolutionary conserved protein kinase that integrates signals from growth factors, nutrients and cellular energy status to regulate cell proliferation, survival, growth and metabolism. mTOR functions have been inferred so far by employing rapamycin, an inhibitor that has therapeutical uses as immunosuppressive and anti cancer agent. However rapamycin is not a general mTOR inhibitor because mTOR is part of at least two distinct multiprotein complexes, one of which is rapamycin insensitive. Thus genetic approaches are needed to study the full spectrum of mTOR functions on growth and metabolism. We analyse mouse lines carrying inactivating mutations in the mTOR signaling pathway. The muscle specific mTOR knockout mice show a dramatic phenotype, as they develop a severe muscular dystrophy providing an animal model of myopathy. These preliminary data indicate a novel link between mTOR signaling deregulation and the development of muscular dystrophy. In addition we have demonstrated that the inactivation of the mTOR substrate S6 kinase 1 (S6K1) is sufficient to trigger muscle atrophy. Our future research aims at identifying the causes of these dystrophic and atrophic phenotypes in mammalian tissues. In collaboration with the Medical Genetics department at the Necker Hospital we are setting up a functional screening of muscle samples from myopathic patients to search for mutations in the mTOR pathways. We will establish primary myoblast cultures from muscle biopsies and evaluate the activities of the distinct mTOR effectors. This rapid analysis should restrict the number of candidate alleles to be sequenced. We predict that defects in the mTOR pathway should underlie a broad range of orphan myopathy diseases, potentially opening new strategies for diagnosis and therapy of skeletal muscle pathologies. Finally we will use gain of function mutants and in vivo gene therapy to address the role of this pathway in cancer progression.
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 muscular dystrophies
- medical and health sciences medical biotechnology genetic engineering gene therapy
- medical and health sciences clinical medicine oncology
- medical and health sciences basic medicine pathology
- medical and health sciences basic medicine medical genetics
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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.
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.
ERC-2007-StG
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.
Host institution
75654 PARIS
France
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.