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SUMMARY:&quot\;Proteostasis imbalance and neurodegenerative diseases&quot\
 ; - Claudio Hetz\, Biomedical Neuroscience Institute\, Santiago\, Chile. 
DTSTART:20160909T120000Z
DTEND:20160909T130000Z
UID:TALK67275@talks.cam.ac.uk
CONTACT:47261
DESCRIPTION:Most neurodegenerative diseases share a common neuropathology\
 , primarily featuring the presence of abnormal protein inclusions containi
 ng specific misfolded proteins. Recent evidence indicates that alteration 
 in organelle function is a common pathological feature of protein misfoldi
 ng disorders. The endoplasmic reticulum (ER) is an essential compartment f
 or protein folding\, maturation\, and secretion. Signs of ER stress have b
 een extensively described in most experimental models of neurological diso
 rders. To cope with ER stress\, cells activate an integrated signaling res
 ponse termed the Unfolded Protein Response (UPR)\, which aims to reestabli
 sh homeostasis through transcriptional upregulation of genes involved in p
 rotein folding\, quality control and degradation pathways. Here we discuss
  our efforts to assess the role of the UPR in brain diseases\, and develop
  gene therapy strategies to alleviate ER stress in specific brain regions.
  Examples in ALS\, Alzheimer´s disease and Parkinson´s disease will be p
 rovided. A new concept is emerging where depending on specific UPR compone
 nt targeted and the disease model tested distinct and even opposite effect
 s can be observed on the pathology.  
LOCATION:Level 7 - Cell Biology in Medicine Lecture\, CIMR
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