title: MicroRNA Dysregulation in Cystic Fibrosis.
abstract: The cystic fibrosis lung is a complex milieu comprising multiple factors that coordinate its physiology. MicroRNAs are regulatory factors involved in most biological processes and it is becoming increasingly clear that they play a key role in the development and manifestations of CF lung disease. These small noncoding RNAs act posttranscriptionally to inhibit protein production. Their involvement in the pathogenesis of CF lung disease stems from the fact that their expression is altered in vivo in the CF lung due to intrinsic and extrinsic factors; to date defective chloride ion conductance, endoplasmic reticulum stress, inflammation, and infection have been implicated in altering endogenous miRNA expression in this setting. Here, the current state-of-the-art and biological consequences of altered microRNA expression in cystic fibrosis are reviewed.
authors.affiliation: Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin, Ireland.
authors.forename: Paul J
authors.initials: PJ
authors.lastname: McKiernan
authors.affiliation: Department of Medicine, Royal College of Surgeons in Ireland, Education and Research Centre, Beaumont Hospital, Dublin, Ireland.
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Greene
mesh_terms: D003550:Cystic Fibrosis / Q000235:genetics*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000502:physiology; D059865:Endoplasmic Reticulum Stress; D005786:Gene Expression Regulation; D006801:Humans; D007113:Immunity, Innate; D035683:MicroRNAs / Q000502:physiology*; D051193:Toll-Like Receptors / Q000502:physiology
chemical_list: C505032:CFTR protein, human; D035683:MicroRNAs; D051193:Toll-Like Receptors; D019005:Cystic Fibrosis Transmembrane Conductance Regulator