P.I. Pascale Belenguer
The impact of visual handicaps has risen in our developed countries with the contemporary means of visual communication and population ageing. Optic nerve atrophies are often due to defects in mitochondria, as Dominant Optic Atrophy (DOA) caused by mutations of the mitochondrial OPA1 protein, a large GTPase involved in mitochondrial dynamics. As there is no cure for DOA, we explore an original therapeutic avenue, inspired by the tendency of viruses to block apoptosis. We focus on a virus-encoded protein called X, demonstrated by our collaborator D. Dunia’s team to protect neurons against diverse neurodegenerative insults. Interestingly, these effects require the localization of X in mitochondria and are correlated to modification of mitochondrial dynamics. We thus test whether X can protect neurons against the consequences of OPA1 deficiencies using both in vitro and in vivo models.
Collaborators : Daniel Dunia (CPTP, Purpan, Toulouse), Bernd Wissinger (University of Tubingen, Germany)