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- Overview of Chaperone Networks
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1. Mapping the molecular chaperone interaction network in yeast
- Prof. Walid A. Houry
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2. The interaction network of the GroEL chaperonin
- Prof. Dr. F. Ulrich Hartl
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3. Human heat shock protein families
- Prof. Herman Kampinga
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4. Extracellular proteostasis: an emerging field
- Prof. Mark Wilson
- Proteasome Networks
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6. Biogenesis of the eukaryotic proteasome
- Prof. Mark Hochstrasser
- Understanding Intrinsically Disordered Proteins in Protein Homeostasis
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7. Intrinsically unstructured proteins: regulation and disease
- Dr. M. Madan Babu
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8. The roles of intrinsic disorder in protein interaction networks
- Prof. Vladimir N. Uversky
- Gene Regulatory Networks and their Role in Protein Homeostasis
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10. Structure, evolution and dynamics of gene regulatory networks
- Dr. M. Madan Babu
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12. Protein-protein interaction networks
- Prof. Peter Csermely
- Protein Homeostasis in the Endoplasmic Reticulum
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13. Role of calnexin and calreticulin in protein homeostasis within the endoplasmic reticulum
- Prof. David B. Williams
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14. The unfolded protein response
- Prof. Kazutoshi Mori
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15. Role of ER stress in cystic fibrosis airway inflammation
- Dr. Carla Maria Pedrosa Ribeiro
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16. The recognition of misfolded glycoproteins in the endoplasmic reticulum
- Dr. David Y. Thomas
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17. Chaperone systems of the endoplasmic reticulum
- Prof. Linda M. Hendershot
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18. The ERAD network
- Prof. Daniel Hebert
- Protein Homeostasis in the Mitochondria
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19. Protein homeostasis in mitochondria: AAA+ chaperones & proteases
- Dr. David A. Dougan
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21. Mitochondrial chaperonin Hsp60: locations, functions and pathology
- Prof. Francesco Cappello
- Prof. Alberto J. L. Macario
- Protein Homeostasis in the Nucleus
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22. Nuclear protein quality control degradation
- Dr. Richard G. Gardner
- Protein Homeostasis in Aging Disease
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23. Protein homeostasis during ageing: C. elegans as a model organism
- Prof. Nektarios Tavernarakis
- Protein Homeostasis in Neurodegeneration
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24. Endoplasmic reticulum stress in neurodegenerative diseases
- Prof. Claudio Soto
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25. Roles for Hsp40 molecular chaperones in protein misfolding disease
- Prof. Douglas M. Cyr
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26. Protein folding in vivo
- Prof. James Bardwell
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27. Protein degradation and defense against neurodegenerative disease 1
- Prof. Alfred Goldberg
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28. Protein degradation and defense against neurodegenerative disease 2
- Prof. Alfred Goldberg
Printable Handouts
Navigable Slide Index
- Introduction
- Cystic fibrosis
- CF airways disease
- Ca levels in CF human airway epithelia cultures
- Ca mobilization in airway epithelia cells
- Apical GPCR activation and Ca signaling
- Apical ER/Ca store expansion
- Summary of earlier studies
- Modeling the CF phenotype
- Mucosal SMM promotes ER/Ca store expansion
- Apical ER/Ca store expansion- Cl- secretion
- Apical ER/Ca store expansion-hyperinflammation
- SMM affects inflammatory mediator production
- SMM affects metabolism and protein synthesis
- Hypothesis: causes of ER/Ca store expansion
- ER unfolded proteins activate UPR pathways
- IRE1 pathway
- Airway epithelial adaptive response hypothesis
- SMM induces XBP-1 mRNA splicing
- SMM induces ER protein chaperone expression
- Increased XBP-1s, BIP and PDI in human CF HBE
- XBP-1 mRNA splicing coupled to IL-8 secretion
- The role of XBP-1 in ER/Ca2+ store expansion (1)
- Spliced XBP-1 promotes ER Ca store expansion
- The role of XBP-1 in ER/Ca2+ store expansion (2)
- DN-XBP-1 inhibit ER Ca store expansion
- The role of spliced XBP-1 in IL-8 secretion
- IRE1 in airway epithelial hyperinflammation
- IRE1 isoforms: IRE1alpha and IRE1beta
- 16HBE14o- cells only express IRE1alpha
- Studying ER Ca store expansion in vivo
- XBP-1 splicing - ER expansion coupling
- XBP-1 splicing is increased in inflamed airways
- Future research directions
- Summary
- 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
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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 March 21, 2025, from https://doi.org/10.69645/JEHJ3806.Export Citation (RIS)
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.
Role of ER stress in cystic fibrosis airway inflammation
Published on February 2, 2012
38 min