The TLR4 regulator miR-21 is downregulated in the paediatric cystic fibrosis airway

摘要: Cystic fibrosis (CF) is a genetic disorder caused by mutations in the cystic transmembrane receptor (CFTR) gene. Defective CFTR function airway creates an environment that exposed to repeated cycles of inflammation and infection, leading progressive decline lung function. MicroRNAs are short nucleotide regulatory sequences bind target mRNAs inhibit their expression. We have previously reported miRNA expression profiling data comparing bronchial brushings from adult CF patients versus age-matched non-CF controls. Several differentially expressed miRNAs since been validated qPCR functionally linked pathophysiology, including those with targets such as CFTR, IL-8 other innate inflammatory mediators. However, likely suffered considerable infection remodelling compared younger patients, this study we sought evaluate differential paediatric samples. Bronchial epithelial cells (BECs) were isolated lungs 6 children (aged 1–6 years). Using high-throughput analysis, 754 between sample groups. The results revealed 21 significantly expressed. From data, miR-21 was selected for further investigation, downregulated BECs relative samples, also CFBE41o– cell line 16HBE14o–. Given roles regulating TLR4 signalling pathway, studies warranted determine if could represent potential early clinical intervention disease.