| Description |
1 online resource (xvi, 528 pages) : illustrations (some color) |
| Series |
Methods in molecular biology, 1940-6029 ; v. 741 |
|
Methods in molecular biology (Clifton, N.J.) ; v. 741. 1064-3745
|
| Contents |
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 [and others] -- 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 [and others] -- 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 [and others] -- 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 [and others] -- 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 [and others] -- 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 [and others] -- 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 [and others] -- Electrophysiological, biochemical, and bioinformatic methods for studying CFTR channel gating and its regulation / Laszlo Csanady [and others] -- 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 |
| Summary |
Despite the many milestones in cystic fibrosis (CF) research, progress towards curing the disease has been slow, and it is increasingly difficult to grasp and use the already wide and still growing range of diverse methods currently employed to study CF so as to understand it in its multidisciplinary nature. Cystic Fibrosis: Diagnosis and Protocols aims to provide the CF research community and related researchers with a very wide range of high-quality experimental tools, as an easy way to grasp and use classical and novel methods applied to cystic fibrosis. Volume I: Approaches to Study and Correct CFTR Defects focuses on the cystic fibrosis transmembrane conductance regulator (CFTR) and its expression, biogenesis, structure, and function in terms of the defects causing CF. Written in the highly successful Methods in Molecular Biology™ series format, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls. Comprehensive and practical, Cystic Fibrosis: Diagnosis and Protocols will provide readers with optimal working tools to address pressing questions in the best technical way, while helping all of us, as a research and clinical community, to move faster hand-in-hand toward unravelling the secrets of this challenging disorder and cure it |
| Analysis |
geneeskunde |
|
medicine |
|
biomedische wetenschappen |
|
biomedicine |
|
humane genetica |
|
human genetics |
|
Medicine (General) |
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Geneeskunde (algemeen) |
| Bibliography |
Includes bibliographical references and index |
| Notes |
Print version record |
| Subject |
Cystic fibrosis.
|
|
Cystic Fibrosis
|
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Cystic Fibrosis -- diagnosis
|
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Cystic fibrosis
|
| Genre/Form |
Laboratory manuals.
|
|
Manuels de laboratoire.
|
| Form |
Electronic book
|
| Author |
Amaral, Margarida D.
|
|
Kunzelmann, Karl
|
| ISBN |
9781617791178 |
|
1617791172 |
|
