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SUMMARY:Novel Mouse Models to Study Mitochondrial Proteostasis in the Nerv
 ous System
DTSTART:20190204T103000
DTEND:20190204T113000
DTSTAMP:20260407T175829Z
UID:9ddc011b902604a0544de1ffb374ba2cf8bdb7943fa5a4f3f2ee4057
CATEGORIES:Conferences - Seminars
DESCRIPTION:Hans-Georg Sprenger\, Max Planck Institute for Biology of Agei
 ng\, Dept. Mitochondrial Proteostasis\, Cologne (D)\nSEMINAR of the LAUSAN
 NE INTEGRATIVE METABOLISM and NUTRITION ALLIANCE (LIMNA)\n\nAbstract:\nDis
 turbances in the morphology and function of mitochondria cause neurologica
 l diseases\, which can affect the central and peripheral nervous system. T
 he i-AAA protease YME1L ensures mitochondrial proteostasis and regulates m
 itochondrial dynamics by processing of the dynamin-like GTPase OPA1. Mutat
 ions in YME1L cause a multi-systemic mitochondriopathy associated with neu
 rological dysfunction and mitochondrial fragmentation but pathogenic mecha
 nisms remained enigmatic. Here\, we report on striking cell-type specific 
 defects in mice lacking YME1L in the nervous system. YME1L-deficient mice 
 manifest ocular dysfunction with microphthalmia and cataracts and at later
  stages of life develop deficiencies in locomotor activity due to specific
  degeneration of spinal cord axons. We demonstrate that YME1L ensures effi
 cient mitochondrial transport in neurons and maintains mitochondrial prote
 ostasis and dynamics in vivo. Additional deletion of Oma1 restores tubular
  mitochondria but deteriorates axonal degeneration in the absence of YME1L
 \, demonstrating that impaired mitochondrial proteostasis rather than mito
 chondrial fragmentation cause trafficking defects and the observed neurolo
 gical dysfunction.\n \n 
LOCATION:AI 1153 https://plan.epfl.ch/?room==AI%201153
STATUS:CONFIRMED
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