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  • Book
    edited by Margarida D. Amaral, Karl Kunzelmann.
    Digital : Springer2011
    Introduction to section I : the relevance of CF diagnostic tools for measuring restoration of CFTR function after therapeutic interventions in human clinical trials / Kris De Boeck and Melissa Ashlock -- High-throughput screening of libraries of compounds to identify CFTR modulators / Nicoletta Pedemonte, Olga Zegarra-Moran, and Luis J.V. Galietta -- Repair of CFTR folding defects with correctors that function as pharmacological chaperones / Tip W. Loo and David M. Clarke -- Use of primary cultures of human bronchial epithelial cells isolated from cystic fibrosis patients for the pre-clinical testing of CFTR modulators / Timothy Neuberger ... [et al.] -- Design of gene therapy trials in CF patients / Jane C. Davies and Eric W.F.W. Alton -- Nasal potential difference measurements to assess CFTR ion channel activity / Steven M. Rowe, John Paul Clancy, and Michael Wilschanski -- Measurement of ion transport function in rectal biopsies / Martin J. Hug ... [et al.] -- Introduction to section II: RNA Methods to approach CFTR expression / Ann Harris -- Quantification of CFTR transcripts / Anabela S. Ramalho, Luka A. Clarke, and Margarida D. Amaral -- Nonsense-mediated mRNA decay and cystic fibrosis / Liat Linde and Batsheva Kerem -- Approaches to study CFTR Pre-mRNA splicing defects / Elisa Goina, Eugenio Fernandez-Alanis, and Franco Pagani -- Impact of microRNA in normal and pathological respiratory epithelia / Lisa Giovannini-Chami ... [et al.] -- Genomic approaches to studying CFTR transcriptional regulation / Christopher J. Ott and Ann Harris -- Introduction to section III : biochemical methods to study CFTR protein / Margarida D. Amaral and Gergely L. Lukacs -- Analysis of CFTR folding and degradation in transiently transfected cells / Diane E. Grove ... [et al.] -- In vitro methods for CFTR biogenesis / Yoshihiro Matsumura, LeeAnn Rooney, and William R. Skach -- Analysis of CFTR interactome in the macromolecular complexes / Chunying Li and Anjaparavanda P. Naren -- Methods to monitor cell surface expression and endocytic trafficking of CFTR in polarized epithelial cells / Jennifer M. Bomberger, William B. Guggino, and Bruce A. Stanton -- Segmental and subcellular distribution of CFTR in the kidney / Francois Jouret, Pierre J. Courtoy, and Olivier Devuyst -- Endocytic sorting of CFTR variants monitored by single-cell fluorescence ratiometric image analysis (FRIA) in living cells / Herve Barriere ... [et al.] -- Introduction to section IV: biophysical methods to approach CFTR structure / Juan L. Mendoza, Andre Schmidt, and Philip J. Thomas -- CFTR three-dimensional structure / Robert C. Ford ... [et al.] -- Molecular modeling tools and approaches for CFTR and cystic fibrosis / Adrian W.R. Serohijos, Patrick H. Thibodeau, and Nikolay V. Dokholyan -- Biochemical and biophysical approaches to probe CFTR structure / Andre Schmidt, Juan L. Mendoza, and Philip J. Thomas -- NMR spectroscopy to study the dynamics and interactions of CFTR / Voula Kanelis, P. Andrew Chong, and Julie D. Forman-Kay -- Introduction to section V : assessment of CFTR function / Karl Kunzelmann -- Application of high-resolution single-channel recording to functional studies of cystic fibrosis mutants / Zhiwei Cai ... [et al.] -- Electrophysiological, biochemical, and bioinformatic methods for studying CFTR channel gating and its regulation / Laszlo Csanady ... [ et al.] -- CFTR regulation by phosphorylation / Rodrigo Alzamora, J Darwin King, and Kenneth R. Hallows -- How to measure CFTR-dependent bicarbonate transport : from single channels to the intact epithelium / Martin J. Hug, Lane L. Clarke, and Michael A. Gray.