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SUMMARY:Primary mitochondrial disorders: from gene finding\, to protein un
 derstanding\,  to treatment
DTSTART:20110510T143000
DTSTAMP:20260406T142511Z
UID:e72c3bd8defbac077668926de81efe6fb2bf483d28b094dc91a16d29
CATEGORIES:Conferences - Seminars
DESCRIPTION:Dr. Massimo ZEVIANI\, MD\, PhD Director\, Unit of Molecular Ne
 urogenetics\, Fondazione Istituto Neurologico “Carlo Besta”\, MILANO\n
 Increased mitochondrial biogenesis has been proposed as a treatment for mi
 tochondrial disease. We tested this hypothesis by activating the AMP-kinas
 e/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 w
 ith 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 therapeu
 tic perspectives for mitochondrial disease.
LOCATION:AI 1153 https://plan.epfl.ch/?room==AI%201153
STATUS:CONFIRMED
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