Journal
BIOLOGICAL CHEMISTRY
Volume 383, Issue 2, Pages 271-282Publisher
WALTER DE GRUYTER & CO
DOI: 10.1515/BC.2002.029
Keywords
endoplasmic reticulum; ER-storage diseases; transcription factors
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The clinical course of Cystic Fibrosis is characterized by recurrent pulmonary infections which ultimately lead to death by respiratory failure. The most common CIF causing mutation, DeltaF508-CFTR, produces an incorrectly folded protein, which accumulates within the endoplasmic reticulum. However, the molecular mechanism by which the DeltaF508-CFTR protein facilitates pulmonary infection and inflammation remains unclear. Here we show that the expression of DeltaF508CFTR causes a constitutive activation of the pro-inflammatory transcription factor NF-kappaB by eliciting an ER stress reaction, the ER-overload response. This endogenous NF-kappaB activation stimulates the transcription of pro-inflammatory cytokines thereby commencing an inflammatory cascade within the CIF lung.
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