CELLSPEX

Multiwavelength cell spectroscopy to define the pathophysiology of mitochondrial disorders in living cells

 Coordinatore MEDICAL RESEARCH COUNCIL 

 Organization address address: NORTH STAR AVENUE POLARIS HOUSE
city: SWINDON
postcode: SN2 1FL

contact info
Titolo: Mrs.
Nome: Irina
Cognome: James
Email: send email
Telefono: 441223000000
Fax: 441223000000

 Nazionalità Coordinatore United Kingdom [UK]
 Totale costo 309˙235 €
 EC contributo 309˙235 €
 Programma FP7-PEOPLE
Specific programme "People" implementing the Seventh Framework Programme of the European Community for research, technological development and demonstration activities (2007 to 2013)
 Code Call FP7-PEOPLE-2013-IIF
 Funding Scheme MC-IIF
 Anno di inizio 2014
 Periodo (anno-mese-giorno) 2014-03-01   -   2016-02-29

 Partecipanti

# participant  country  role  EC contrib. [€] 
1    MEDICAL RESEARCH COUNCIL

 Organization address address: NORTH STAR AVENUE POLARIS HOUSE
city: SWINDON
postcode: SN2 1FL

contact info
Titolo: Mrs.
Nome: Irina
Cognome: James
Email: send email
Telefono: 441223000000
Fax: 441223000000

UK (SWINDON) coordinator 309˙235.20

Mappa


 Word cloud

Esplora la "nuvola delle parole (Word Cloud) per avere un'idea di massima del progetto.

instrumentation    drive    mitochondrial    therapies    cells    oxphos    clinical    bioenergetic    mitochondria    expertise    molecular    function    force    disease    motive    cell    proton    disorders    mutations    living    biochemical    transfer   

 Obiettivo del progetto (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.'

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