Defects of Secretion in Cystic Fibrosis (eBook)

Acknowledgments 6
Contributing Authors 7
Preface 11
Chapter 1 OUTSIDE NEURONS/ INSIDE EPITHELIA: NOVEL ACTIVATION OF CFTR CL ANDHCO3 CONDUCTANCES 14
Chapter 2 ROLE OF CFTR AND OTHER ION CHANNELS IN CYSTIC FIBROSIS 35
Chapter 3 ION CHANNELS IN THE APICAL MEMBRANE: ROLE OF ELECTRICAL COUPLING ON TRANSEPITHELIAL TRANSPORT 54
Chapter 4 ION CHANNELS IN SECRETORY GRANULES OF THE PANCREAS: MOLECULAR IDENTIFICATION AND THEIR ROLE IN REGULATED SECRETION 63
Chapter 5 EPITHELIAL TRANSPORT AND INTRACELLULAR TRAFFICKING: PHYSIOLOGY AND PATHOPHYSIOLOGY 76
Chapter 6 KINASES, CELL VOLUME, AND THE REGULATION OF CHLORIDE CHANNELS 82
Chapter 7 THE CLCAS: PROTEINS WITH ION CHANNEL, CELL ADHESION AND TUMOR SUPPRESSOR FUNCTIONS 91
Chapter 8 IS INTERVENTION IN INOSITOL PHOSPHATE SIGNALING A USEFUL THERAPEUTIC OPTION FOR CYSTIC FIBROSIS? 111
Chapter 9 AN INOSITOL PHOSPHATE ANALOG, INO- 4995, NORMALIZES ELECTROPHYSIOLOGY IN CF AIRWAY EPITHELIA 123
Chapter 10 VITAMIN C AND FLAVONOIDS POTENTIATE CFTR CL TRANSPORT IN HUMAN AIRWAY EPITHELIA 136
Chapter 11 AIRWAY GLYCOCONJUGATES SECRETED IN CYSTIC FIBROSIS AND SEVERE CHRONIC AIRWAY INFLAMMATION RELATIONSHIP WITH PSEUDOMONAS AERUGINOSA 152
Chapter 12 BIOSYNTHESIS AND SECRETION OF MUCINS, ESPECIALLY THE MUC2 MUCIN, IN RELATION TO CYSTIC FIBROSIS 175