Explore the words cloud of the mtDNA-CURE project. It provides you a very rough idea of what is the project "mtDNA-CURE" about.
The following table provides information about the project.
|Coordinator Country||Sweden [SE]|
|Total cost||2˙500˙000 €|
|EC max contribution||2˙500˙000 € (100%)|
1. H2020-EU.1.1. (EXCELLENT SCIENCE - European Research Council (ERC))
|Duration (year-month-day)||from 2018-01-01 to 2022-12-31|
Take a look of project's partnership.
|1||KAROLINSKA INSTITUTET||SE (STOCKHOLM)||coordinator||2˙500˙000.00|
|2||MAX-PLANCK-GESELLSCHAFT ZUR FORDERUNG DER WISSENSCHAFTEN EV||DE (Munich)||participant||0.00|
This proposal describes a series of powerful experimental strategies to develop a completely novel treatment for mtDNA mutation disease based on identifying unknown mechanisms controlling mtDNA replication. Several hundred different mtDNA mutations affect tRNA genes and impair mitochondrial translation leading to human disease. There is typically heteroplasmy with a mixture of wild-type and mutated mtDNA, and the mutations are acting in a “recessive” (loss of function) way. Very high levels of mutated mtDNA are needed to cause disease in affected patients whereas maternal relatives with high, but sub-threshold levels of mutated mtDNA are completely healthy. The corollary of these observations is that even a small increase of wild-type mtDNA may efficiently counteract disease in affected patients. This hypothesis will be validated by a series of genetic experiments with mice harbouring single pathogenic mtDNA mutations. Furthermore, novel factors controlling mtDNA replication will be identified. In particular, we will elucidate the formation and function of the mammalian displacement (D) loop, which provides a switch between abortive and genome length mtDNA replication. This very fundamental problem in mammalian mitochondrial biology has remained unsolved for decades, but I feel that the innovative experimental strategies I present in this proposal are very powerful and should have a fair chance of being successful. In any circumstance, the project will provide important molecular insights into novel mechanisms relevant for mammalian mtDNA replication. Over the years I have been strongly convinced that congruent results from in vivo and in vitro studies are needed to obtain reliable mechanistic insights and this project is therefore based on the close integration of biochemistry, advanced proteomics and state-of-the-art mouse and fly genetics. Finally, I describe a powerful large-scale screening approach to develop small molecular stimulators of mtDNA replication.
|year||authors and title||journal||last update|
R. Filograna, C. Koolmeister, M. Upadhyay, A. Pajak, P. Clemente, R. Wibom, M. L. Simard, A. Wredenberg, C. Freyer, J. B. Stewart, N. G. Larsson
Modulation of mtDNA copy number ameliorates the pathological consequences of a heteroplasmic mtDNA mutation in the mouse
published pages: eaav9824, ISSN: 2375-2548, DOI: 10.1126/sciadv.aav9824
|Science Advances 5/4||2019-11-22|
Are you the coordinator (or a participant) of this project? Plaese send me more information about the "MTDNA-CURE" project.
For instance: the website url (it has not provided by EU-opendata yet), the logo, a more detailed description of the project (in plain text as a rtf file or a word file), some pictures (as picture files, not embedded into any word file), twitter account, linkedin page, etc.
Send me an email (firstname.lastname@example.org) and I put them in your project's page as son as possible.
Thanks. And then put a link of this page into your project's website.
The information about "MTDNA-CURE" are provided by the European Opendata Portal: CORDIS opendata.