MicroRNA Dysregulation in Cystic Fibrosis

Positive Expiratory Pressure (PEP) versus Conventional Chest Physiotherapy in Pediatric Patients with Acute Exacerbation of Cystic Fibrosis

Background Pulmonary involvement is the main cause of mortality in cystic fibrosis (CF). Airway clearance techniques are non-pharmacological complement options for CF patients. The aim of this study was to evaluate the short-term outcome of airway cleaning treatment in patients with cystic fibrosis in a children's hospital. Materials and Methods This clinical trial study conducted on 40 CF pati...

Molecular screening of R117H mutation in non caucasian cystic fibrosis patients in the north of Iran

Cystic fibrosis is an autosomal recessive disease caused by a wide spectrum of mutations in the gene encoding for the cystic fibrosis transmembrane conductance regulator protein. These mutations that correlate with different phenotypes, vary in their frequency and distribution in different populations. In this study missense mutation R117H that associated with the different clinical symptoms wa...

A Peptide Nucleic Acid against MicroRNA miR-145-5p Enhances the Expression of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) in Calu-3 Cells.

Peptide nucleic acids (PNAs) are very useful tools for gene regulation at different levels, but in particular in the last years their use for targeting microRNA (anti-miR PNAs) has provided impressive advancements. In this respect, microRNAs related to the repression of cystic fibrosis transmembrane conductance regulator (CFTR) gene, which is defective in cystic fibrosis, are of great importanc...

Regulation of Cystic Fibrosis Transmembrane Conductance Regulator by MicroRNA-145, -223, and -494 Is Altered in DF508 Cystic Fibrosis Airway Epithelium

Analysis of CFTR Gene Mutations in Children with Cystic Fibrosis, First Report from North-East of Iran

 Objective(s):  More than 1500 registered mutations in cystic fibrosis transmembrane regulator (CFTR) gene are responsible for dysfunction of an ion channel protein and a wide spectrum of clinical manifestations in patients with cystic fibrosis (CF). This study was performed to investigate the frequency of a number of well-known CFTR mutations in North Eastern Iranian CF patients. Material and...