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1. Introduction
2. Cystic fibrosis
3. CF airways disease
4. Ca levels in CF human airway epithelia cultures
5. Ca mobilization in airway epithelia cells
6. Apical GPCR activation and Ca signaling
7. Apical ER/Ca store expansion
8. Summary of earlier studies
9. Modeling the CF phenotype
10. Mucosal SMM promotes ER/Ca store expansion
11. Apical ER/Ca store expansion- Cl- secretion
12. Apical ER/Ca store expansion-hyperinflammation
13. SMM affects inflammatory mediator production
14. SMM affects metabolism and protein synthesis
15. Hypothesis: causes of ER/Ca store expansion
16. ER unfolded proteins activate UPR pathways
17. IRE1 pathway
18. Airway epithelial adaptive response hypothesis
19. SMM induces XBP-1 mRNA splicing
20. SMM induces ER protein chaperone expression
21. Increased XBP-1s, BIP and PDI in human CF HBE
22. XBP-1 mRNA splicing coupled to IL-8 secretion
23. The role of XBP-1 in ER/Ca2+ store expansion (1)
24. Spliced XBP-1 promotes ER Ca store expansion
25. The role of XBP-1 in ER/Ca2+ store expansion (2)
26. DN-XBP-1 inhibit ER Ca store expansion
27. The role of spliced XBP-1 in IL-8 secretion
28. IRE1 in airway epithelial hyperinflammation
29. IRE1 isoforms: IRE1alpha and IRE1beta
30. 16HBE14o- cells only express IRE1alpha
31. Studying ER Ca store expansion in vivo
32. XBP-1 splicing - ER expansion coupling
33. XBP-1 splicing is increased in inflamed airways
34. Future research directions
35. Summary
36. Acknowledgments

Topics Covered

• Pathophysiology: abnormal rheology of airway secretions
• Impaired mucociliary clearance
• Airway obstruction
• Persistent lung infections and inflammation
• Respiratory failure
• Progressive lung disease as the main cause of death
• Role of ER stress responses in CF airways inflammation
• Studies on the mechanism of ER stress activation-mediated airway cytokine secretion

Links

Series:

• Protein Homeostasis

Categories:

• Biochemistry
• Cell Biology
• Clinical Practice

Therapeutic Areas:

• Immunology & Inflammation
• Respiratory Diseases

Talk Citation

Ribeiro, C.M.P. (2012, February 2). Role of ER stress in cystic fibrosis airway inflammation [Video file]. In The Biomedical & Life Sciences Collection, Henry Stewart Talks. Retrieved April 19, 2025, from https://doi.org/10.69645/JEHJ3806.
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Publication History

• Published on February 2, 2012

Financial Disclosures

• Dr. Carla Maria Pedrosa Ribeiro has not informed HSTalks of any commercial/financial relationship that it is appropriate to disclose.