Mitochondrial Bioenergetics in Disease: Toward a Mitochondrial Therapy of Muscular Dystrophies and Beyond
Paolo Bernardi, MD
University of Padova
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Originally presented September 15, 2009
The hypothesis that Ca2+-dependent mitochondrial dysfunction could play a role in muscular dystrophies has been put forward 30 years ago. An important development in this field has been the demonstration that the mitochondrial permeability transition plays a key role in the pathogenesis of muscular dystrophy in collagen VI deficiencies both in mouse models and in patients with Ullrich Congenital Muscular Dystrophy and Bethlem Myopathy. This finding, which is opening new therapeutic perspectives for these diseases, was made possible by a careful dissection of mitochondrial function in muscle fibers from mice and in cultured cells from patients, and by the analysis of mitochondrial responses to various treatments and drugs.
Join us for this 45-minute webinar where we will recapitulate these steps, discuss potential problems, and illustrate new developments in the field of mitochondrial DNA diseases.
In this webinar we will discuss:
- How the mitochondrial membrane potential responds to respiratory inhibitors and oligomycin in healthy and diseased cells.
- The nature of the permeability transition and the bioenergetic consequences of its occurrence.
- The mechanism through which the MPT is desensitized by ligands of cyclophilin D like cyclosporin A.
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