Background Ataluren was developed to restore functional protein production in genetic disorders
caused by nonsense mutations, which are the cause of cystic fibrosis in 10% of patients…

Chronic inflammation of the lung, as a consequence of persistent bacterial infections by
several opportunistic pathogens represents the main cause of mortality and morbidity in cystic …

Introduction In this study we investigated the effects of the Cystic Fibrosis Transmembrane
conductance Regulator (CFTR) gene variants on the composition of faecal microbiota, in …

Bdellovibrio bacteriovorus is a predator bacterial species found in the environment and within
the human gut, able to attack Gram-negative prey. Cystic fibrosis (CF) is a genetic disease …

microRNAs (miRNAs) are a class of regulatory small non-coding molecules that control gene
expression at post-transcriptional level. Deregulation of miRNA functions affects a variety of …

Objectives To date, PROPELLER MRI, a breathing-motion-insensitive technique, has not
been assessed for cystic fibrosis (CF) lung disease. We compared this technique to CT for …

Inflammatory lung disease is a primary cause of morbidity and mortality in cystic fibrosis (CF).
Mechanisms of unresolved acute inflammation in CF are not completely known, although …

N-3-oxo-dodecanoyl-homoserine lactone (3OC 12 -HSL) is the main quorum sensing (QS)
signal produced by the human pathogen Pseudomonas aeruginosa, a major cause of hard-to…

Background The effect of complex alleles in cystic fibrosis (CF) is poorly defined for the lack
of functional studies. Objectives To describe the genotype–phenotype correlation and the …

MicroRNAs (miRNAs) have recently emerged as important gene regulators in Cystic Fibrosis
(CF), a common monogenic disease characterized by severe infection and inflammation, …