作者: Florence Sonneville , Manon Ruffin , Loïc Guillot , Nathalie Rousselet , Philippe Le Rouzic
DOI: 10.1016/J.AJPATH.2014.12.022
关键词:
摘要: The molecular basis of cystic fibrosis (CF) is a mutation-related defect in the epithelial–cell chloride channel called CF transmembrane conductance regulator (CFTR). This alters ion transport and impairs water across cell membrane. Marked clinical heterogeneity occurs even among patients carrying same mutation CFTR gene. Recent studies suggest that such could be related to epigenetic factors and/or miRNAs, which are small noncoding RNAs modulate expression various proteins via post-transcriptional inhibition gene expression. In respiratory system, it has been shown dysregulation miRNAs participate lead pathogenicity several diseases. airways, recent have proposed may disease progression by affecting production either or dysregulated lung. Herein, we provide an overview showing how pathology efforts develop miRNA-based treatments consider as biomarkers. identification involved opens up new avenues toward targeting selected components CF, independently from mutation.