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Table of Contents
Vol. 4, No. 1-2, 1994
Issue release date: 1994
Section title: Original Paper
Cell Physiol Biochem 1994;4:10–18
(DOI:10.1159/000154705)

Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus Oocytes

Carroll T.P. · Mclntosh I. · Egan M.E. · Zeitlin P.L. · Cutting G.R. · Guggino W.B.
Departments of Physiology, Pediatrics and Medicine, Johns Hopkins University School of Medicine, Baltimore, Md., USA

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Article / Publication Details

First-Page Preview
Abstract of Original Paper

Accepted: February 02, 1993
Published online: September 04, 2008
Issue release date: 1994

Number of Print Pages: 9
Number of Figures: 0
Number of Tables: 0

ISSN: 1015-8987 (Print)
eISSN: 1421-9778 (Online)

For additional information: http://www.karger.com/CPB

Abstract

We report that Xenopus oocytes injected with wild-type cystic fibrosis transmembrane regulator (CFTR) RNA exhibit cAMP-regulated Cl currents. Injection of CFTR RNA bearing Rl 1 7H, a mutation associated with mild disease, induces cAMP-regulated Cl currents. The magnitudes of the currents induced by R117H were consistently lower than those induced by expression of wild-type CFTR. Western blotting with anti-CFTR antibody confirms that protein was expressed in oocytes injected with either normal or the mutant form of CFTR mRNA. The rank order of single channel open channel probabilities of wild-type and mutant CFTR Cl channels is wild-type > R117H. These data demonstrate that mutations in the transmembrane region affect CFTR channel properties and are consistent with the hypothesis that CF disease severity may be correlated with reduced cAMP-regulated Cl conductances of mutant CFTR.

© 1994 S. Karger AG, Basel


Article / Publication Details

First-Page Preview
Abstract of Original Paper

Accepted: February 02, 1993
Published online: September 04, 2008
Issue release date: 1994

Number of Print Pages: 9
Number of Figures: 0
Number of Tables: 0

ISSN: 1015-8987 (Print)
eISSN: 1421-9778 (Online)

For additional information: http://www.karger.com/CPB


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