Primary mitochondrial disorders: from gene finding, to protein understanding, to treatment
Event details
| Date | 10.05.2011 |
| Hour | 14:30 |
| Speaker | Dr. Massimo ZEVIANI, MD, PhD Director, Unit of Molecular Neurogenetics, Fondazione Istituto Neurologico “Carlo Besta”, MILANO |
| Location | |
| Category | Conferences - Seminars |
Increased mitochondrial biogenesis has been proposed as a treatment for mitochondrial disease. We tested this hypothesis by activating the AMP-kinase/PGC-1α pathway in three cytochrome c oxidase defective mouse models: a knockout for Surf1, a knockout/knockin for Sco2, and a muscle-restricted knockout for Cox15. We first showed partial correction of COX deficiency in our models by either PGC-1α transgenic overexpression, or treatment with the AMPK agonist AICAR. We then observed remarkable recovery of motor performance up to normal levels in AICAR-treated Sco2KO/KI mice. These and other results that will be presented during the seminar open new therapeutic perspectives for mitochondrial disease.
Links
Practical information
- General public
- Free
Contact
- (dans le cadre du Lausanne Integrative Metabolism & Nutrition Alliance - LIMNA)