CRISTOPA1

Extending the Optic atrophy 1 dependent cristae remodeling: from models to a rationale for therapy of autosomal dominant optic atrophy

 Coordinatore UNIVERSITA DEGLI STUDI DI PADOVA 

 Organization address address: VIA 8 FEBBRAIO 2
city: PADOVA
postcode: 35122

contact info
Titolo: Dr.
Nome: Emanuela
Cognome: Pavia
Email: send email
Telefono: 390498000000
Fax: 390498000000

 Nazionalità Coordinatore Italy [IT]
 Totale costo 100˙000 €
 EC contributo 100˙000 €
 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-CIG
 Funding Scheme MC-CIG
 Anno di inizio 2013
 Periodo (anno-mese-giorno) 2013-10-01   -   2017-09-30

 Partecipanti

# participant  country  role  EC contrib. [€] 
1    UNIVERSITA DEGLI STUDI DI PADOVA

 Organization address address: VIA 8 FEBBRAIO 2
city: PADOVA
postcode: 35122

contact info
Titolo: Dr.
Nome: Emanuela
Cognome: Pavia
Email: send email
Telefono: 390498000000
Fax: 390498000000

IT (PADOVA) coordinator 100˙000.00

Mappa


 Word cloud

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

metabolism    functions    function    regulates    adoa    apoptosis    mutations    opa    cristae    multiple    vivo    mitochondrial    autophagy    mitochondria    optic    protein    atrophy   

 Obiettivo del progetto (Objective)

'Autosomal dominant optic atrophy (ADOA) is caused by mutations in Optic Atrophy 1 (OPA1), a dynamin-related protein of the inner mitochondrial membrane. During the last years, we clarified that OPA1 is a multifunctional protein participating in genetically distinct pathways of mitochondrial fusion and cristae remodelling, both impaired by pathogenetic mutations. We extended our investigation on the (dys)function of OPA1 and our preliminary results indicate that (i) OPA1 is a key modulator of apoptosis and autophagy in vivo; (ii) OPA1 is a master regulator of mitochondrial cristae architecture, impacting on respiratory chain supercomplex assembly and mitochondrial metabolism; (iii) increased autophagy in axons of retinal ganglion cells carrying pathogenic OPA1 depletes them of mitochondria; (iv) OPA1 resides in multimolecular complexes that comprise potential keyregulators of its multiple functions. We therefore hypothesize that by engaging in interactions with different partners, OPA1 regulates mitochondrial functions. Its mutations increase autophagy and susceptibility to apoptosis, especially in RGCs. These multiple regulatory points offer several potential targets for therapeutic strategies that can interfere with the natural course of the disease. In order to verify our hypothesis, we plan to address: (i) how OPA1 regulates mitochondrial metabolism from the cristae; (ii) how changes in OPA1 levels and function impinge on autophagy, especially in RGC; (iii) if the changes in mitochondrial metabolism and autophagy can be exploited therapeutically in vitro and in vivo. This integrated approach aims at unraveling the pathogenesis of ADOA, and therefore to pave the way for its treatment. At the same time, we expect to clarify how mitochondria participate in key cellular metabolic and quality control processes.'

Altri progetti dello stesso programma (FP7-PEOPLE)

SIGNLEARNSPEAK (2011)

Examining the relationship between linguistic- and non-linguistic cognitive processes in deaf children's vocabulary development in spoken and signed vocabulary

Read More  

HICATLHC (2014)

Phenomenology of Heavy Ion Collisions at RHIC and the LHC

Read More  

QTOPDEV (2014)

Topological Quantum Devices

Read More