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Biological Chemistry

Editor-in-Chief: Brüne, Bernhard

Editorial Board: Buchner, Johannes / Lei, Ming / Ludwig, Stephan / Thomas, Douglas D. / Turk, Boris / Wittinghofer, Alfred

IMPACT FACTOR 2018: 3.014
5-year IMPACT FACTOR: 3.162

CiteScore 2018: 3.09

SCImago Journal Rank (SJR) 2018: 1.482
Source Normalized Impact per Paper (SNIP) 2018: 0.820

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Volume 383, Issue 2


ΔF508-CFTR Causes Constitutive NF-κB Activation through an ER-Overload Response in Cystic Fibrosis Lungs

A. Knorre / M. Wagner / H.-E. Schaefer / W.H. Colledge / H.L. Pahl
Published Online: 2005-06-01 | DOI: https://doi.org/10.1515/BC.2002.029


The clinical course of Cystic Fibrosis is characterized by recurrent pulmonary infections which ultimately lead to death by respiratory failure. The most common CF causing mutation, δF508-CFTR, produces an incorrectly folded protein, which accumulates within the endoplasmic reticulum. However, the molecular mechanism by which the δF508-CFTR protein facilitates pulmonary infection and inflammation remains unclear. Here we show that the expression of δF508-CFTR causes a constitutive activation of the proinflammatory transcription factor NFκB by eliciting an ER stress reaction, the ERoverload response. This endogenous NFκB activation stimulates the transcription of proinflammatory cytokines thereby commencing an inflammatory cascade within the CF lung.

About the article

Published Online: 2005-06-01

Published in Print: 2002-02-15

Citation Information: Biological Chemistry, Volume 383, Issue 2, Pages 271–282, ISSN (Print) 1431-6730, DOI: https://doi.org/10.1515/BC.2002.029.

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