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Cystic Fibrosis: A Trilogy of Biochemistry, Physiology, and Therapy

Subject Area(s):  Human Biology and DiseaseBiochemistry

Edited by John R. Riordan, The University of North Carolina at Chapel Hill; Richard C. Boucher, The University of North Carolina at Chapel Hill; Paul M. Quinton, University of California, San Diego

© 2013 • 340 pp., illus. (40 4C; 15 B&W), index
Hardcover •
ISBN  978-1-936113-34-7

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Cystic fibrosis is caused by mutations in the CFTR gene, which encodes an ion channel protein that regulates anion movement across the epithelial membranes of the lungs, pancreas, and other organs. In cystic fibrosis patients, anion transport is impeded, causing sticky, viscous mucus to build up and clog these vital organs.

Written and edited by experts in the field, this collection from Cold Spring Harbor Perspectives in Medicine provides an in-depth examination of cystic fibrosis biology and treatment strategies. Contributors examine the structure and dynamics of CFTR, its normal physiological roles in the airway and digestive epithelia, and how those operations are impaired in patients with cystic fibrosis. The numerous CFTR mutations and how they alter the expression, synthesis, processing, and function of CFTR in cystic fibrosis and other CFTR-related disorders are considered, as are disease-modifying genes that influence disease severity.

This volume includes discussions of therapy and treatment strategies for cystic fibrosis, ranging from airway clearance techniques and pancreatic enzyme replacements to the modulation of CFTR and related ion transport pathways. It will be an essential reference for molecular and cellular biologists, physiologists, and clinicians interested in understanding the biological basis of the disease and the search for effective therapies.


The Cystic Fibrosis Gene: A Molecular Genetic Perspective
Lap-Chee Tsui and Ruslan Dorfman
Assessing the Disease-Liability of Mutations in CFTR
Claude Ferec and Garry R. Cutting
The CFTR Ion Channel: Gating, Regulation, and Anion Permeation
Tzyh-Chang Hwang and Kevin L. Kirk
Cystic Fibrosis Transmembrane Conductance Regulator (ABCC7) Structure
John F. Hunt, Chi Wang, and Robert C. Ford
Dynamics Intrinsic to Cystic Fibrosis Transmembrane Conductance Regulator Function and Stability
P. Andrew Chong, Pradeep Kota, Nikolay V. Dokholyan, and Julie D. Forman-Kay
The Influence of Genetics on Cystic Fibrosis Phenotypes
Michael R. Knowles and Mitchell Drumm
Status of Fluid and Electrolyte Absorption in Cystic Fibrosis
M.M. Reddy and M. Jackson Stutts
Physiology of Epithelial Chloride and Fluid Secretion
Raymond A. Frizzell and John W. Hanrahan
Mechanisms of Bicarbonate Secretion: Lessons from the Airways
Robert J. Bridges
Transepithelial Bicarbonate Secretion: Lessons from the Pancreas
Hyun Woo Park and Min Goo Lee
CFTR, Mucins, and Mucus Obstruction in Cystic Fibrosis
Silvia M. Kreda, C. William Davis, and Mary Callaghan Rose
Supramolecular Dynamics of Mucus
Pedro Verdugo
Perspectives on Mucus Properties and Formation Lessons from the Biochemical World
Daniel Ambort, Malin E.V. Johansson, Jenny K. Gustafsson, Anna Ermund, and Gunnar C. Hansson
Structure and Function of the Mucus Clearance System of the Lung
Brenda M. Button and Brian Button
The Cystic Fibrosis Airway Microbiome
Susan V. Lynch and Kenneth D. Bruce
The Cystic Fibrosis of Exocrine Pancreas
Michael Wilschanski and Ivana Novak
The Cystic Fibrosis Intestine
Robert C. De Lisle and Drucy Borowitz
Cystic Fibrosis Transmembrane Regulator Correctors and Potentiators
Steven M. Rowe and Alan S. Verkman
Antibiotic and Anti-Inflammatory Therapies for Cystic Fibrosis
James F. Chmiel, Michael W. Konstan, and J. Stuart Elborn
New Pulmonary Therapies Directed at Targets Other than CFTR
Scott H. Donaldson and Luis Galietta

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