Cystic Fibrosis Research Advances

Single Cell RNAseq Reveals A Critical Role of Chloride Channels in Airway Development

This study uses single-cell RNAseq to identify chloride channel roles in airway development, showing their impact on differentiation and disease-like defects.

Whole genome sequencing enables definitive diagnosis of Cystic Fibrosis and Primary Ciliary Dyskinesia

The paper uses whole genome sequencing to identify disease-causing variants in non-coding regions of genes, confirming diagnoses of Cystic Fibrosis and Primary Ciliary Dyskinesia.

Whole genome sequencing reveals the emergence of a Pseudomonas aeruginosa shared strain sub-lineage among patients treated within a single cystic fibrosis centre

The paper uses whole genome sequencing to show that M3L7, a multidrug-resistant sub-type of AUST-02, emerged as a shared strain among CF patients in a single centre, with evidence of both continuous infection and cross-infection.

Mutations in C11ORF70 cause primary ciliary dyskinesia with randomization of left/right body asymmetry due to outer and inner dynein arm defects

PP-2, a src-kinase inhibitor, is a potential corrector for F508del-CFTR in cystic fibrosis

The paper identifies PP-2, a src-kinase inhibitor, as a novel corrector for ΔF508-CFTR in cystic fibrosis through in silico screening and experimental validation.

Modified hCFTR mRNA restores normal lung function in a mouse model of cystic fibrosis

This study shows that modified hCFTR mRNA delivered as nanoparticles improves lung function in a mouse model of cystic fibrosis.