Cystic Fibrosis

Chapter 1 Introduction to Section I: The Relevance of CF Diagnostic Tools for Measuring Restoration of CFTR Function After Therapeutic Interventions in Human Clinical Trials

Chapter 2 High-Throughput Screening of Libraries of Compounds to Identify CFTR Modulators

Chapter 3 Repair of CFTR Folding Defects with Correctors that Function as Pharmacological Chaperones

Chapter 4 Use of Primary Cultures of Human Bronchial Epithelial Cells Isolated from Cystic Fibrosis Patients for the Pre-clinical Testing of CFTR Modulators

Chapter 5 Design of Gene Therapy Trials in CF Patients

Chapter 6 Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity

Chapter 7 Measurement of Ion Transport Function in Rectal Biopsies

Chapter 8 Introduction to Section II: RNA Methods to Approach CFTR Expression

Chapter 9 Quantification of CFTR Transcripts

Chapter 10 Nonsense-Mediated mRNA Decay and Cystic Fibrosis

Chapter 11 Approaches to Study CFTR Pre-mRNA Splicing Defects

Chapter 12 Impact of MicroRNA in Normal and Pathological Respiratory Epithelia

Chapter 13 Genomic Approaches to Studying CFTR Transcriptional Regulation

Chapter 14 Introduction to Section III: Biochemical Methods to Study CFTR Protein

Chapter 15 Analysis of CFTR Folding and Degradation in Transiently Transfected Cells

Chapter 16 In Vitro Methods for CFTR Biogenesis

Chapter 17 Analysis of CFTR Interactome in the Macromolecular Complexes

Chapter 18 Methods to Monitor Cell Surface Expression and Endocytic Trafficking of CFTR in Polarized Epithelial Cells

Chapter 19 Segmental and Subcellular Distribution of CFTR in the Kidney

Chapter 20 Endocytic Sorting of CFTR Variants Monitored by Single-Cell Fluorescence Ratiometric Image Analysis (FRIA) in Living Cells

Chapter 21 Introduction to Section IV: Biophysical Methods to Approach CFTR Structure

Chapter 22 CFTR three-dimensional structure.

Chapter 23 Molecular Modeling Tools and Approaches for CFTR and Cystic Fibrosis

Chapter 24 Biochemical and Biophysical Approaches to Probe CFTR Structure

Chapter 25 NMR Spectroscopy to Study the Dynamics and Interactions of CFTR

Chapter 26 Introduction to Section V: Assessment of CFTR Function

Chapter 27 Application of High-Resolution Single-Channel Recording to Functional Studies of Cystic Fibrosis Mutants

Chapter 28 Electrophysiological, Biochemical, and Bioinformatic Methods for Studying CFTR Channel Gating and Its Regulation

Chapter 29 CFTR Regulation by Phosphorylation

Chapter 30 How to Measure CFTR-Dependent Bicarbonate Transport: From Single Channels to the Intact Epithelium