The cystic fibrosis transmembrane regulator forms macromolecular complexes with PDZ domain scaffold proteins.

Research output: Contribution to journalArticle

Abstract

Cystic fibrosis transmembrane regulator (CFTR), an epithelial Cl- channel defective in cystic fibrosis, is localized at the apical membrane of epithelial cells. CFTR interacts with a number of ion channels (outwardly rectifying chloride channels, epithelial sodium channels, and inwardly rectifying potassium channels), protein kinases A and C, and putative protein phosphatases and ATP transporters. These data suggest that CFTR may exist in macromolecular complexes with these and other scaffolding proteins at the apical membrane. PDZ domain proteins have been shown to localize and cluster CFTR in the Golgi and at the plasma membrane. This review highlights the role of PDZ domain proteins in regulating the trafficking and processing of CFTR.

Original languageEnglish (US)
Pages (from-to)28-32
Number of pages5
JournalProceedings of the American Thoracic Society
Volume1
Issue number1
StatePublished - 2004

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PDZ Domains
Macromolecular Substances
Cystic Fibrosis
Proteins
Inwardly Rectifying Potassium Channel
Epithelial Sodium Channels
Chloride Channels
Membranes
Phosphoprotein Phosphatases
Cyclic AMP-Dependent Protein Kinases
Ion Channels
Protein Kinase C
Adenosine Triphosphate
Epithelial Cells
Cell Membrane

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Physiology
  • Cell Biology

Cite this

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abstract = "Cystic fibrosis transmembrane regulator (CFTR), an epithelial Cl- channel defective in cystic fibrosis, is localized at the apical membrane of epithelial cells. CFTR interacts with a number of ion channels (outwardly rectifying chloride channels, epithelial sodium channels, and inwardly rectifying potassium channels), protein kinases A and C, and putative protein phosphatases and ATP transporters. These data suggest that CFTR may exist in macromolecular complexes with these and other scaffolding proteins at the apical membrane. PDZ domain proteins have been shown to localize and cluster CFTR in the Golgi and at the plasma membrane. This review highlights the role of PDZ domain proteins in regulating the trafficking and processing of CFTR.",
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AB - Cystic fibrosis transmembrane regulator (CFTR), an epithelial Cl- channel defective in cystic fibrosis, is localized at the apical membrane of epithelial cells. CFTR interacts with a number of ion channels (outwardly rectifying chloride channels, epithelial sodium channels, and inwardly rectifying potassium channels), protein kinases A and C, and putative protein phosphatases and ATP transporters. These data suggest that CFTR may exist in macromolecular complexes with these and other scaffolding proteins at the apical membrane. PDZ domain proteins have been shown to localize and cluster CFTR in the Golgi and at the plasma membrane. This review highlights the role of PDZ domain proteins in regulating the trafficking and processing of CFTR.

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