Abstract

Long non-coding RNAs (lncRNA) were previously found deregulated in many pathological conditions, including pulmonary diseases. It is not known if their expression changes during enhanced inflammatory processes in the airways in paediatric diseases. Therefore, we compared the expression of lncRNA airway and peripheral profiles between pulmonary exacerbation (PE) and stable period in cystic fibrosis (CF) pediatric patients. We included four CF patients during PE and in stable condition. In all subjects, we performed a microarray analysis of transcriptome in samples of sputum and whole blood. Sputum samples were collected during spontaneous expectoration and prepared by the addition of Sputolysin. RNA extraction was done using Nucleospin RNA blood kit from blood samples and Nucleospin RNA/protein kit for sputum samples. The expression profile of lncRNAs was done using SurePrint G3 Human Gene Expression microarrays. The differential gene expression analysis of lncRNA was done using GeneSpring software v.14.9.1. We found 127 lncRNAs expressed in sputum and 193 lncRNAs in whole blood samples, 6 lncRNAs were detected in both sample types. Lnc-RASA1-7, lnc-SLC44A5-1, lnc-CHAF1B-2 and lnc-GSR-1 were downregulated, while lnc-MRC2-1 showed elevated expression during PE in two kinds of samples. For lnc-PPP4R1-8, we observed it was upregulated in blood, but showed reduced expression in sputum during PE. <b>Conclusions:</b> Expression of lncRNAs profile changes during cystic fibrosis pulmonary exacerbation, suggesting their possible role in regulating disease course. The study was supported by the Polish National Science Centre, grant no. 2016/22/E/NZ5/00383 (A. Szczepankiewicz).

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