HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Guggino, W. B. Search for Related Content PubMed PubMed Citation Articles by Guggino, W. B.

The Cystic Fibrosis Transmembrane Regulator Forms Macromolecular Complexes with PDZ Domain Scaffold Proteins

Department of Physiology, Johns Hopkins University School of Medicine, Baltimore, Maryland

Correspondence and requests for reprints should be addressed to William B. Guggino, Ph.D., Department of Physiology, Johns Hopkins University School of Medicine, Baltimore, MD 21205. E-mail: wguggino{at}bs.jhmi.edu

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.

Key Words: chloride • regulation • trafficking • processing • channel

This article has been cited by other articles:

				M. Dayem, C. Basquin, V. Navarro, P. Carrier, R. Marsault, P. Chang, S. Huc, E. Darrouzet, S. Lindenthal, and T. Pourcher Comparison of expressed human and mouse sodium/iodide symporters reveals differences in transport properties and subcellular localization J. Endocrinol., April 1, 2008; 197(1): 95 - 109. [Abstract] [Full Text] [PDF]

				T.-Y. Chen and T.-C. Hwang CLC-0 and CFTR: Chloride Channels Evolved From Transporters Physiol Rev, April 1, 2008; 88(2): 351 - 387. [Abstract] [Full Text] [PDF]

				C. Mazzochi, D. J. Benos, and P. R. Smith Interaction of epithelial ion channels with the actin-based cytoskeleton Am J Physiol Renal Physiol, December 1, 2006; 291(6): F1113 - F1122. [Abstract] [Full Text] [PDF]