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Regulation of mRNA turnover in cystic fibrosis lung disease

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Cystic fibrosis (CF) is an autosomal recessive disease due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, F508del‐CFTR being the most frequent mutation. The CF lung is characterized by a hyperinflammatory phenotype and is regulated by multiple factors that coordinate its pathophysiology. In CF the expression of CFTR as well as proinflammatory genes are regulated at the level of messenger RNA (mRNA) stability, which subsequently affect translation. These mechanisms are mediated by inflammatory RNA‐binding proteins as well as small endogenous noncoding microRNAs, in coordination with cellular signaling pathways. These regulatory factors exhibit altered expression and function in vivo in the CF lung, and play a key role in the pathophysiology of CF lung disease. In this review, we have described the role of mRNA stability and associated regulatory mechanisms in CF lung disease. WIREs RNA 2017, 8:e1408. doi: 10.1002/wrna.1408 This article is categorized under: RNA Turnover and Surveillance > Regulation of RNA Stability Regulatory RNAs/RNAi/Riboswitches > Regulatory RNAs RNA in Disease and Development > RNA in Disease
Factors that regulate messenger RNA (mRNA) turnover. The mRNA 3′‐untranslated region (UTR) of proinflammatory genes and cystic fibrosis transmembrane conductance regulator (CFTR) contains AU‐rich elements (ARE motifs) (represented by green boxes, top panel) and is subject to the complex mechanism of ARE‐specific sequential degradation (bottom panel). AUBPs, ARE‐binding proteins.
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MicroRNAs (miRs) regulate cystic fibrosis (CF) disease phenotype. The mutant F508del‐CFTR (cystic fibrosis transmembrane conductance regulator) leads to altered expression of miRs. These miRs can affect F508del‐CFTR expression and function as well as F508del‐CFTR‐dependent inflammation.
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RNA Turnover and Surveillance > Regulation of RNA Stability
Regulatory RNAs/RNAi/Riboswitches > Regulatory RNAs
RNA in Disease and Development > RNA in Disease

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