Defects in Secretion of Cystic Fibrosis presents an overview on current research from leading experts in North America and Europe. This update on cystic fibrosis provides in depth original work as well as review material on many of the relevant physiological and molecular topics in the field. Subjects covered include the interplay of the various epithelial ion channels, the underlying intracellular signal transduction, mucus secretion, and novel approaches to develop drugs against cystic fibrosis. This book brings together physicians, physiologists, and other scientists involved in basic research, from molecular biology to drug design and introduces novel investigative and therapeutic aspects of secretion disorders relevant in cystic fibrosis and related diseases.

This book will be of interest to Molecular biologists, physiologists, scientists working in pharmaceutical research and drug developement, physicians and researchers in Cystic fibrosis and related diseases.

Zusammenfassung

Defects in Secretion of Cystic Fibrosis presents an overview on current research from leading experts in North America and Europe. This update on cystic fibrosis provides in depth original work as well as review material on many of the relevant physiological and molecular topics in the field. Subjects covered include the interplay of the various epithelial ion channels, the underlying intracellular signal transduction, mucus secretion, and novel approaches to develop drugs against cystic fibrosis. This book brings together physicians, physiologists, and other scientists involved in basic research, from molecular biology to drug design and introduces novel investigative and therapeutic aspects of secretion disorders relevant in cystic fibrosis and related diseases.

This book will be of interest to Molecular biologists, physiologists, scientists working in pharmaceutical research and drug developement, physicians and researchers in Cystic fibrosis and related diseases.

Inhalt
Outside Neurons/Inside Epithelia: Novel Activation of CFTR Cl? and HCO3 ? Conductances.- Role of CFTR and Other Ion Channels in Cystic Fibrosis.- Ion Channels in the Apical Membrane: Role of Electrical Coupling on Transepithelial Transport.- Ion Channels in Secretory Granules of the Pancreas: Molecular Identification and Their Role in Regulated Secretion.- Epithelial Transport and Intracellular Trafficking: Physiology and Pathophysiology.- Kinases, Cell Volume, and the Regulation of Chloride Channels.- The CLCAs: Proteins with Ion Channel, Cell Adhesion and Tumor Suppressor Functions.- Is Intervention in Inositol Phosphate Signaling a Useful Therapeutic Option for Cystic Fibrosis?.- An Inositol Phosphate Analog, INO-4995, Normalizes Electrophysiology in CF Airway Epithelia.- Vitamin C and Flavonoids Potentiate CFTR Cl Transport in Human Airway Epithelia.- Airway Glycoconjugates Secreted in Cystic Fibrosis and Severe Chronic Airway Inflammation Relationship with Pseudomonas aeruginosa.- Biosynthesis and Secretion of Mucins, Especially the MUC2 Mucin, in Relation to Cystic Fibrosis.