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Related Concept Videos

Export of Misfolded Proteins out of the ER01:32

Export of Misfolded Proteins out of the ER

After folding, the ER assesses the quality of secretory and membrane proteins. The correctly folded proteins are cleared by the calnexin cycle for transport to their final destination, while misfolded proteins are held back in the ER lumen. The ER chaperones attempt to unfold and refold the misfolded proteins but sometimes fail to achieve the correct native conformation. Such terminally misfolded proteins are then exported to the cytosol by ER-associated degradation or ERAD pathway for...

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Analysis of Protein Folding, Transport, and Degradation in Living Cells by Radioactive Pulse Chase
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NHE-RF1 protein rescues DeltaF508-CFTR function.

Florian Bossard1, Amal Robay, Gilles Toumaniantz

  • 1Institut National de la Santé et de la Recherche Médicale Unité 533, l' Institut du Thorax, Faculté de Médecine, Nantes, France.

American Journal of Physiology. Lung Cellular and Molecular Physiology
|January 24, 2007
PubMed
Summary

NHE-RF1 protein enhances the delivery of DeltaF508-cystic fibrosis transmembrane conductance regulator (CFTR) to the cell surface, improving chloride channel activity in epithelial cells. This finding offers a potential therapeutic strategy for cystic fibrosis treatment.

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Area of Science:

  • Cell Biology
  • Molecular Biology
  • Biochemistry

Background:

  • Cystic fibrosis (CF) is characterized by inefficient trafficking of the DeltaF508-CFTR protein to the plasma membrane.
  • PDZ-containing proteins are crucial for the polarized expression of plasma membrane ion transporters.
  • NHE-RF1 is a PDZ-containing protein known to bind the carboxy terminus of CFTR.

Purpose of the Study:

  • To investigate whether the PDZ-containing protein NHE-RF1 can rescue the expression and activity of DeltaF508-CFTR at the apical membrane of epithelial cells.
  • To determine if NHE-RF1's interaction with CFTR is essential for this rescue effect.

Main Methods:

  • Intranuclear injection of plasmids encoding DeltaF508-CFTR and NHE-RF1 into A549 and MDCK cells.
  • Functional assay of DeltaF508-CFTR channel activity using the SPQ fluorescent probe.
  • Immunocytochemical analysis to assess protein localization.
  • Use of antisense and sense oligonucleotides to investigate the role of NHE-RF1.

Main Results:

  • Coexpression of DeltaF508-CFTR with NHE-RF1 significantly increased basal and forskolin-activated chloride conductances.
  • This enhancement was lost when DeltaF508-CFTR lacked its terminal 13 amino acids or when NHE-RF1 function was inhibited by antisense oligonucleotides.
  • Immunocytochemistry confirmed that NHE-RF1 specifically directed DeltaF508-CFTR to the apical plasma membrane.

Conclusions:

  • NHE-RF1 can rescue the apical membrane expression and chloride channel activity of DeltaF508-CFTR.
  • Modulating the expression of CFTR protein partners, such as NHE-RF1, represents a promising therapeutic strategy for cystic fibrosis.