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SUMMARY:SPECIAL BMI SEMINAR - ER proteostasis unbalance: from aging to neu
 rodegenerative diseases
DTSTART:20180927T160000
DTEND:20180927T170000
DTSTAMP:20260924T111122Z
UID:3527904920b651604d4530ba4643d622e212682dbb535ae4f93dd1c1
CATEGORIES:Conferences - Seminars
DESCRIPTION:Claudio Hetz\, PhD Biomedical Neuroscience Institute (BNI)\, F
 aculty of Medicine\, Chile\nThe clinical manifestation of neurodegenerativ
 e diseases is initiated by the selective alteration in the functionality o
 f distinct neuronal populations. At the molecular level\, most neurodegene
 rative diseases share common molecular signatures including the accumulati
 on of misfolded proteins in the brain. Alteration in the buffering capacit
 y of the proteostasis network is proposed as one of the triggering steps l
 eading to abnormal protein aggregation\, which is also a central hallmarks
  of brain aging.  The endoplasmic reticulum (ER) is a major node of the p
 roteostasis network altered in brain diseases and during aging. ER stress 
 triggers a signaling reaction known as the unfolded protein response (UPR)
 \, which aims restoring proteostasis through the induction of adaptive pro
 grams or the activation of cell death programs when damage is chronic and 
 cannot be repaired. Here we discuss our efforts to assess the significance
  of the UPR to brain aging and its contribution to disease\, in addition t
 o develop gene therapy strategies to alleviate ER stress. A new concept is
  emerging where depending on specific UPR component targeted and the disea
 se model tested distinct and even opposite effects can be observed on the 
 pathology. \n \nThis work was funded by FONDAP program 15150012\, Millen
 nium Institute P09-015-F\, Michael J Fox Foundation for Parkinson´s Resea
 rch – Target Validation grant No 9277\, ALS Therapy Alliance 2014-F-059\
 , Muscular Dystrophy Association 382453\, FONDEF ID16I10223\, FONDEF D11E1
 007\, FONDECYT no. 1140549\, ALSRP Therapeutic Idea Award AL150111\, US Of
 fice of Naval Research-Global (ONR-G) N62909-16-1-2003\, U.S. Air Force Of
 fice of Scientific Research FA9550-16-1-0384\, European Commission R&D\, a
 nd MSCA-RISE #734749 and CONICYT-Brazil 441921/2016-7.\n \n 
LOCATION:SV 1717 https://plan.epfl.ch/?room==SV%201717
STATUS:CONFIRMED
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