"ER proteostasis disturbances in neurodegenerative diseases"
Event details
| Date | 24.11.2015 |
| Hour | 13:30 › 14:30 |
| Speaker | Prof. Claudio Hetz - Biomedical Neuroscience Institute (BNI), Faculty of Medicine, University of Chile and Harvard School of Public Health, Boston, USA. |
| Location | |
| Category | Conferences - Seminars |
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 including ALS and Parkinson’s disease, and develop gene therapy strategies to alleviate ER stress in specific brain regions. 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 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.
This 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.
Practical information
- General public
- Free
Organizer
- Neurodegenerative Studies Laboratory (LEN)
Contact
- Dr Bernard Schneider