Proteins belonging to the ATP-binding cassette superfamily couple ATP binding and hydrolysis at conserved nucleotide-binding domains (NBDs) to diverse cellular functions. Most superfamily members are transporters, while cystic fibrosis transmembrane conductance regulator (CFTR), alone, is an ion channel. Despite this functional difference, recent results have suggested that CFTR shares a common...

The gastroduodenal mucosa is a dynamic barrier restricting entry of gastric acid and other potentially hostile luminal contents. Mucosal HCO3(-) is a key element in preventing epithelial damage, and knowledge about HCO3(-) transport processes, including the role of the cystic fibrosis transmembrane conductance regulator channel, and their neurohumoral control are in rapid progress.

Abstract Purpose of Review To review the role ocular surface epithelial (corneal and conjunctival) ion transporters in pathogenesis treatment dry eye disease (DED). Recent Findings Currently, anti-inflammatory agents are mainstay DED treatment, though there several development that target transport proteins on surface, acting by pro-secretory or anti-absorptive mechanisms to increase tear fluid...

Cystic fibrosis (CF) is caused by CFTR (cystic fibrosis transmembrane conductance regulator) gene mutations. We ascertained five patients with a novel complex CFTR allele, with two mutations, H939R and H949L, inherited in cis in the same exon of CFTR gene, and one different mutation per patient inherited in trans in a wide population of 289 Caucasian CF subjects from South Italy. The genotype-p...

Nasal carriage is a major risk factor for Staphylococcus aureus infection, especially for methicillin-resistant strains (MRSA). Using a mouse model of nasal carriage, we have compared several S. aureus strains and demonstrated increased colonization levels by MRSA in cystic fibrosis transmembrane conductance regulator-deficient mice and Toll-like receptor 2 (TLR2)-deficient mice but not TLR4-de...

OBJECTIVE To study expression of cystic fibrosis transmembrane conductance regulator (CFTR) in human endometrium. METHODS The expression of CFTR mRNA and protein from 50 samples of normal cyclic human endometrium was examined by in situ hybridization, immunohistochemistry and Western blotting respectively. RESULTS CFTR mRNA and protein expressions were only detected in the endometrial gland...

In 1983, Paul Quinton showed that the chloride permeability of sweat duct epithelium was essentially abolished in cystic fibrosis (CF) (1). Shortly after, cyclic adenosine monophosphate (cAMP)–dependent transport of chloride across airway epithelia was also shown to be greatly reduced in CF, and the defect was localized to the apical membrane (2, 3). Therefore, it was to be anticipated that, wi...

Cystic fibrosis (CF) is characterized by an abnormality in cAMP-regulated chloride transport that results from a primary defect in the protein product of the CF gene, the CF transmembrane conductance regulator (CFTR). In this report, antibodies against CFTR peptides were used to localize the CFTR protein in human pancreas. An affinity purified antibody (alpha-1468) raised against a synthetic CF...

Increasing evidence suggests heterogeneity in the molecular pathogenesis of cystic fibrosis (CF). Mutations such as deletion of phenylalanine at position 508 (delta F508) within the cystic fibrosis transmembrane conductance regulator (CFTR), for example, appear to cause disease by abrogating normal biosynthetic processing, a mechanism which results in retention and degradation of the mutant pro...

Vasoactive intestinal peptide (VIP), a neuropeptide, controls multiple functions in exocrine tissues, including inflammation, and relaxation of airway and vascular smooth muscles, and regulates CFTR-dependent secretion, which contributes to mucus hydration and local innate defense of the lung. We had previously reported that VIP stimulates the VPAC1 receptor, PKCϵ signaling cascade, and increas...