Cystic Fibrosis : Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects /

Bibliographic Details
Corporate Author: SpringerLink (Online service)
Other Authors: Amaral, Margarida D. (Editor), Kunzelmann, Karl. (Editor)
Format: eBook
Language:English
Published: Totowa, NJ : Humana Press : Imprint: Humana, 2011.
Edition:1st ed. 2011.
Series:Methods in Molecular Biology, 741
Subjects:
Table of Contents:
  • Introduction to Part I: The Relevance of CF Diagnostic Tools for Measuring Restoration of CFTR Function after Therapeutic Interventions in Human Clinical Trials
  • High-Throughput Screening of Libraries of Compounds to Identify CFTR Modulators
  • Repair of CFTR Folding Defects with Correctors that Function as Pharmacological Chaperones
  • Use of Primary Cultures of Human Bronchial Epithelial Cells Isolated from Cystic Fibrosis Patients for the Pre-Clinical Testing of CFTR Modulators
  • Design of Gene Therapy Trials in CF Patients
  • Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity
  • Measurement of Ion Transport Function in Rectal Biopsies
  • Introduction to Part II: RNA Methods to Approach CFTR Expression
  • Quantification of CFTR Transcripts
  • Nonsense-Mediated mRNA Decay and Cystic Fibrosis
  • Approaches to Study CFTR Pre-mRNA Splicing Defects
  • Impact of MicroRNA in Normal and Pathological Respiratory Epithelia
  • Genomic Approaches to Studying CFTR Transcriptional Regulation
  • Introduction to Part III: Biochemical Methods to Study CFTR Protein
  • Analysis of CFTR Folding and Degradation in Transiently Transfected Cells
  • In Vitro Methods for CFTR Biogenesis
  • Analysis of CFTR Interactome in the Macromolecular Complexes
  • Methods to Monitor Cell Surface Expression and Endocytic Trafficking of CFTR in Polarized Epithelial Cells
  • Segmental and Subcellular Distribution of CFTR in the Kidney
  • Endocytic Sorting of CFTR Variants Monitored by Single Cell Fluorescence Ratio Image Analysis (FRIA) in Living Cells
  • Introduction to Part IV: Biophysical Methods to Approach CFTR Structure
  • CFTR Three-Dimensional Structure
  • Molecular Modeling Tools and Approaches for CFTR and Cystic Fibrosis
  • Biochemical and Biophysical Approaches to Probe CFTR Structure
  • NMR Spectroscopy to Study the Dynamics and Interactions of CFTR
  • Introduction to Part V: Assessment of CFTR Function
  • Application of High-Resolution Single-Channel Recording to Functional Studies of Cystic Fibrosis Mutants
  • Electrophysiological, Biochemical, and Bioinformatic Methods for Studying CFTR Channel Gating and Its Regulation
  • CFTR Regulation by Phosphorylation
  • How to Measure CFTR-Dependent Bicarbonate Transport: From Single Channels to the Intact Epithelium.