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The discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) as the gene product mutated in cystic fibrosis and its identification as a cAMP-dependent protein kinase-regulated Cl∼ channel have provided important

The idea that cystic fibrosis (CF) results from dysregulation of ion channels, including the epithelial Na+channel (ENaC), evolved from observations made before the cloning of the CF gene in 1989. Multiple laboratories had reported that protein kinase A (PKA) stimulated a Cl∼ channel called ...

One approach for understanding the regulation of CFTR and determining how CFTR regulates the activity of other ion channels is to identify CFTR-associated proteins at the apical membrane of airway epithelia. The development of improved methods for studying protein interactions has l ...

After the identification of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, numerous expression studies verified that CFTR is a cAMP-dependent chloride channel (1–3). Given that chloride impermeability is the signature of CF-affected epithelium, these d ...

Cystic fibrosis transmembrane conductance regulator (CFTR) is aphosphorylation and ATP-dependent Cl∼ channel. It is predominantly expressed in the apical membrane of epithelial cells. The presence and function of CFTR in a cell is sensitively measured using electrophysiolog ...

CFTR, the chloride ion channel encoded by the gene mutated in cystic fibrosis patients, has been the subject of intense investigation since its discovery in 1989 (1). A member of the ATP Binding Cassette (ABC) superfamily, the CFTR channel possesses two nucleotide-binding domains (NBDs) that h ...

The importance of impaired HCO3 secretion in the pathophysiology of the pancreas of cystic fibrosis (CF) patients has been well documented and known for many years (1). Studies in 1990s from the Welsh (2) and Boucher (3) laboratories suggested that HCO3 secretion may also be impaired in the airways ...

The cystic fibrosis transmembrane conductance regulator (CFTR) is a tightly regulated anion channel expressed in epithelial and other cells. It conducts chloride and bicarbonate ions through the plasma membrane and also influences the activity of other channels and transporters ...

Transepithelial chloride secretion requires the activation of both apical membrane chloride channels and basolateral membrane potassium channels. Chloride channels are needed for the apical membrane exit of chloride and potassium channels are needed to repolarize the cell a ...

Studies of purified, reconstituted cystic fibrosis transmembrane conductance regulator (CFTR) protein have proven invaluable in defining those functions that are intrinsic to the CFTR molecule. Reconstitution of purified CFTR protein in planar lipid bilayers provided dir ...

Patients with cystic fibrosis (CF) succumb to airway infection and inflammation, and determining the extent and nature of the infecting agents, as well as the extent and nature of the inflammatory response, is critical to understanding the pathophysiology of the disease and how to intervene ...

Progressive obstructive pulmonary disease accounts for the majority of morbidity and mortality in cystic fibrosis (CF) (1). Chronic bacterial bronchopul-monary colonization and infection plays a major role in this progression. While the lungs of persons with CF are normal in utero, ma ...

Enzyme-Linked Immunosorbent Assays (ELISAs) have had many thousands of applications over the past 25 yr The technique has been used in all fields of pure and applied aspects of biology, in particular it forms the backbone of diagnostic tech- niques The systems used to perform ELISAs make use of anti ...

Correlative microscopy is a hybrid method that allows the localization of events observed under visible, ultraviolet, or infrared light, at molecular and submolecular levels, combining two microscopy techniques. However, the main limitation of correlative microscopy is to deve ...

With the completion of the Arabidopsis thaliana genome sequencing project the next major challenge is the assignment of biological functions to the more than 25,000 genes. Reverse genetics is a powerful tool to elucidate gene function in Arabidopsis. Increasingly sophisticated gen ...

Quantum dots have potential applications in the biomedical field and especially in bioimaging owing to their tunable fluorescent properties. Although many phenotypic studies have been carried out using QDs on different cell lines, only very few of them involved the analysis of the effect ...

The ability to target proteins with nanostructures and/or nanodevices in vivo is important for understanding and controlling their biological function. Quantum dots (QDs) serve as an ideal model nanostructure due to their superior optical properties that permit visual confirma ...

Detailed protocols for the preparation of CdSe nanocrystals coated with a multishell structure of ZnS and their surface modification based on the successive ion layer adsorption and reaction method are described. The first phase of the synthesis produces a hydrophobic surface consi ...

Immobilization of functional proteins such as enzymes on solid surfaces produces a variety of effects ranging from the reversal and strong inhibition to the enhancement of protein stability and function. Such effects are protein-dependent and are affected by the physical and chemical ...

Nanotechnology provides new tools for gene expression analysis that allow for sensitive and specific characterization of prognostic signatures related to cancer. Cancer is a multigenic complex disease where multiple gene loci contribute to the phenotype. The ability to simulta ...