You are here: Home :: Area NEM :: Life sciences :: Biochemistry

A. Knorre, M. Wagner, H.-E. Schaefer, W.H. Colledge, H.L. Pahl

?F508-CFTR Causes Constitutive NF-?B Activation through an ER-Overload Response in Cystic Fibrosis Lungs

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, ?F508-CFTR, produces an incorrectly folded protein, which accumulates within the endoplasmic reticulum. However, the molecular mechanism by which the ?F508-CFTR protein facilitates pulmonary infection and inflammation remains unclear. Here we show that the expression of ?F508-CFTR causes a constitutive activation of the proinflammatory transcription factor NF?B by eliciting an ER stress reaction, the ERoverload response. This endogenous NF?B activation stimulates the transcription of proinflammatory cytokines thereby commencing an inflammatory cascade within the CF lung.

Biological Chemistry, Walter de Gruyter

Print ISSN: 1431-6730
Volume: 383, 02/2002
Pages: 271 - 282

Show full article (external site)

Show all available items of this journal

 

Area NEM