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SUMMARY:"ER proteostasis disturbances in neurodegenerative diseases"
DTSTART:20151124T133000
DTEND:20151124T143000
DTSTAMP:20260406T144423Z
UID:ff50ad9a18e201f86de38126ed8bbfb9278d9c66a4bb4a7bb8451389
CATEGORIES:Conferences - Seminars
DESCRIPTION:Prof. Claudio Hetz - Biomedical Neuroscience Institute (BNI)\,
  Faculty of Medicine\, University of Chile and Harvard School of Public He
 alth\, Boston\, USA.\nMost neurodegenerative diseases share a common neuro
 pathology\, primarily featuring the presence of abnormal protein inclusion
 s containing specific misfolded proteins. Recent evidence indicates that a
 lteration in organelle function is a common pathological feature of protei
 n misfolding disorders. The endoplasmic reticulum (ER) is an essential com
 partment for protein folding\, maturation\, and secretion. Signs of ER str
 ess have been extensively described in most experimental models of neurolo
 gical disorders. To cope with ER stress\, cells activate an integrated sig
 naling response termed the Unfolded Protein Response (UPR)\, which aims to
  reestablish homeostasis through transcriptional upregulation of genes inv
 olved in protein folding\, quality control and degradation pathways. Here 
 we discuss our efforts to assess the role of the UPR in brain diseases inc
 luding ALS and Parkinson’s disease\, and develop gene therapy strategies
  to alleviate ER stress in specific brain regions. A new concept is emergi
 ng where depending on specific UPR component targeted and the disease mode
 l tested distinct and even opposite effects can be observed on the patholo
 gy. \nThis work was funded by FONDECYT 1100176\, Millennium Institute No.
  P09-015-F\, Ring Initiative ACT1109\, FONDEF grant No. D11I1007\, CONICYT
  grant USA2013-0003\, ALS Therapy Alliance\, The Michael J. Fox Foundation
  for Parkinson Research\, and the Frick Foundation.
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STATUS:CONFIRMED
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