Teams

The BioDynaMit team gathers great levels of expertise focusing on mitochondria (cell biology, genetics and bioenergetics). Combining quantitative phase imaging and machine learning the group aim to develop new approaches to quantify mitochondrial dynamics to elucidate molecular defects underpinning neuropathies associated with mitochondrial fusion deficiency.

Due to their key energetic role, the localization of mitochondria at intracellular sites of high-energy demand is crucial to maintain cell energy metabolism. In muscle, mitochondria are embedded between myofibrils that consume ATP during contraction. Likewise, in neurons, mitochondria are transported and accumulate in synapses to provide the energy required to maintain and regulate neurotransmission. Their intracellular distribution depends on their mobility and their highly plastic morphology, which results from balanced fusion and fission processes. Beyond its role in maintaining proper mitochondrial morphology and subcellular location, mitochondrial dynamics is key to maintain mitochondrial genome, which is essential to ensure proper OXPHOS assembly and activity. During the past two decades, a significant amount of relevant data has been obtained on the proteins involved in mitochondrial fusion/fission dynamics, notably several dynamin related proteins (DRPs: DRP1, OPA1, MFN1, MFN2). Interestingly, pathogenic mutations in OPA1 and MFN2 cause neuropathies ranging from mild to severe form, causing dominant optic atrophy or peripheral neuropathies as Charcot-Marie-Tooth (CMT) type 2A disease.