"Proteostasis imbalance and neurodegenerative diseases"

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• Claudio Hetz, Biomedical Neuroscience Institute, Santiago, Chile.
• Friday 09 September 2016, 13:00-14:00
• Level 7 - Cell Biology in Medicine Lecture, CIMR.

If you have a question about this talk, please contact gm522.

If you would like to meet with the speaker contact Giovanna Mallucci gm522@cam.ac.uk or Stefan Marciniak sjm20@cam.ac.uk

Most neurodegenerative diseases share a common neuropathology, primarily featuring the presence of abnormal protein inclusions containing specific misfolded proteins. Recent evidence indicates that alteration in organelle function is a common pathological feature of protein misfolding disorders. The endoplasmic reticulum (ER) is an essential compartment for protein folding, maturation, and secretion. Signs of ER stress have been extensively described in most experimental models of neurological disorders. To cope with ER stress, cells activate an integrated signaling response termed the Unfolded Protein Response (UPR), which aims to reestablish homeostasis through transcriptional upregulation of genes involved in protein 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 provided. A new concept is emerging where depending on specific UPR component targeted and the disease model tested distinct and even opposite effects can be observed on the pathology.

This talk is part of the BRC Seminar Series series.

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