In the organization and editing of this book, the attempt was to combine some of the current perspectives on the basic functional properties of the CFTR channel with information regarding the link between defects in this channel and cystic fibrosis (CF). The issues contained were selected for review because of their timeliness and importance to the CFTR and CF fields.

The first four chapters address issues related to the function and regulation of the CFTR channel and its role in controlling fluid and electrolyte transport across epithelial tissues. The next three chapters cover the lack of a generally accepted view of how altered CFTR activity causes cystic fibrosis. Then the final two chapters discuss possible strategies for repairing or circumventing the CFTR defect in CF patients.

CFTR continues to surprise us with its intriguing properties and functional diversity. The Cystic Fibrosis Transmembrane Conductance Regulator provides a glimpse into some of these properties and insights into several of the remaining questions regarding the biology and pathophysiology of this fascinating and critically important ion channel.

Contents

1. Anion Conduction by CFTR: Mechanisms and Models
2. Phosphatase Regulation of CFTR
3. Control of Membrane Transport by the Cystic Fibrosis Transmembrane Regulator (CFTR)
4. CFTR Interacting Proteins
5. The CFTR Gene and Genetics of Cystic Fibrosis
6. CFTR and Airway Pathophysiology
7. Inflammatory Responses in the Cystic Fibrosis Lung
8. Pharmacology of F508-CFTR Biosynthesis
9. Gene Therapy for Cystic Fibrosis

Index

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