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1. Introduction
2. Neurodegenerative disease (1)
3. Neurodegenerative disease (2)
4. Neurodegenerative disease (3)
5. What causes neurodegenerative diseases?
6. Misfolded aggregates deposited in the brain
7. Role of protein aggregates in disease
8. Research strategy
9. Prion diseases
10. Two conformations of the prion protein
11. Brain alterations in prion diseases
12. Experimental scrapie model in mice
13. Sequence of events in scrapie pathogenesis
14. ERp57 upregulation
15. ERp57 and PrPSc correlation in the brain
16. Upregulation of other ER chaperones
17. ER-stress and UPR in human CJD
18. The UPR pathway
19. XBP-1 conditional KO did not affect prion disease
20. PrP interaction with ER chaperones in brain
21. PrPSc induces neuronal death in vitro
22. PrPSc induces ER-stress in vitro
23. Prion infection sensitizes cells to ER-stress
24. Calcium release comes from the ER
25. Hypothesis: Prion formation leads to ER stress
26. Calcineurin properties
27. Signaling pathways
28. Calcineurin is implicated in PrPSc toxicity
29. A novel treatment for prion diseases
30. Treatment improves symptoms (behavior)
31. Treatment increases animal survival
32. FK506 effect on calcineurin and its targets
33. Treatment decreases neuronal death
34. Treatment decreases neurodegeneration
35. Conclusions
36. Acknowledgment
37. Former lab members

Topics Covered

• Neurodegenerative disease
• Misfolded aggregates deposited in the brain
• Research strategy
• Prion diseases
• Two conformations of the prion protein
• Experimental scrapie model in mice
• Upregulation of ER chaperones
• ER-stress and UPR in human CJD
• PrP interaction with ER chaperones in brain
• PrPSc induction of ER-stress and neuronal death
• Calcineurin properties
• A novel treatment for prion diseases

Links

Series:

• Protein Homeostasis

Categories:

• Biochemistry
• Cell Biology
• Neuroscience

Therapeutic Areas:

• Neurology

Talk Citation

Soto, C. (2012, February 2). Endoplasmic reticulum stress in neurodegenerative diseases [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved April 15, 2025, from https://doi.org/10.69645/KWGE4872.
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Publication History

• Published on February 2, 2012

Financial Disclosures

• Prof. Claudio Soto has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.