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Main description:
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 (TM) 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.
Contents:
Part I: Strategies to Correct the Basic Defect in CF and Assess Efficacy in Human Clinical Trials
1. Introduction to Part 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
2. High-Throughput Screening of Libraries of Compounds to Identify CFTR Modulators
Nicoletta Pedemonte, Olga Zegarra-Moran, and Luis J.V. Galietta
3. Repair of CFTR Folding Defects with Correctors that Function as Pharmacological Chaperones
Tip W. Loo and David M. Clarke
4. Use of Primary Cultures of Human Bronchial Epithelial Cells Isolated from Cystic Fibrosis Patients for the Pre-Clinical Testing of CFTR Modulators
Tim Neuberger, Bill Burton, Heather Clark, and Fredrick Van Goor
5. Design of Gene Therapy Trials in CF Patients
Jane C. Davies and Eric W.F.W. Alton
6. Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity
Steven M. Rowe, Jean-Paul Clancy, and Michael Wilschanski
7. Measurement of Ion Transport Function in Rectal Biopsies
Martin J. Hug, Nico Derichs, Inez Bronsveld, and Jean Paul Clancy
Part II: RNA Methods to Approach CFTR Expression
8. Introduction to Part II: RNA Methods to Approach CFTR Expression
Ann Harris
9. Quantification of CFTR Transcripts
Anabela S. Ramalho, Luka A. Clarke, and Margarida D. Amaral
10. Nonsense-Mediated mRNA Decay and Cystic Fibrosis
Liat Linde and Batsheva Kerem
11. Approaches to Study CFTR Pre-mRNA Splicing Defects
Elisa Goina, Eugenio Fernandez-Alanis, and Franco Pagani
12. Impact of MicroRNA in Normal and Pathological Respiratory Epithelia
Lisa Giovannini-Chami, Nathalie Grandvaux, Laure-Emmanuelle Zaragosi, Karine Robbe-Sermesant, Brice Marcet, Bruno Cardinaud, Christelle Coraux, Yves Berthiaume, Rainer Waldmann, Bernard Mari, and Pascal Barbry
13. Genomic Approaches to Studying CFTR Transcriptional Regulation
Christopher J. Ott and Ann Harris
Part III: CFTR Protein Biogenesis, Folding, Degradation, and Traffic
14. Introduction to Part III: Biochemical Methods to Study CFTR Protein
Margarida D. Amaral and Gergely L. Lukacs
15. Analysis of CFTR Folding and Degradation in Transiently Transfected Cells
Diane E. Grove, Meredith F. N. Rosser, Richard L. Watkins, and Douglas M. Cyr
16. In Vitro Methods for CFTR Biogenesis
Yoshihiro Matsumura, LeeAnn Rooney, and William R. Skach
17. Analysis of CFTR Interactome in the Macromolecular Complexes
Chunying Li and Anjaparavanda P. Naren
18. 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
19. Segmental and Subcellular Distribution of CFTR in the Kidney
Francois Jouret, Pierre J. Courtoy, and Olivier Devuyst
20. Endocytic Sorting of CFTR Variants Monitored by Single Cell Fluorescence Ratio Image Analysis (FRIA) in Living Cells
Herve Barriere, Pirjo Apaja, Tsukasa Okiyoneda, and Gergely L. Lukacs
Part IV: CFTR Structure
21. Introduction to Part IV: Biophysical Methods to Approach CFTR Structure
Juan L. Mendoza, Andre Schmidt, and Philip J. Thomas
22. CFTR Three-Dimensional Structure
Robert C. Ford, James Birtley, Mark F. Rosenberg, and Liang Zhang
23. Molecular Modeling Tools and Approaches for CFTR and Cystic Fibrosis
Adrian W.R. Serohijos, Patrick H. Thibodeau, and Nikolay V. Dokholyan
24. Biochemical and Biophysical Approaches to Probe CFTR Structure
Andre Schmidt, Juan L. Mendoza, and Philip J. Thomas
25. NMR Spectroscopy to Study the Dynamics and Interactions of CFTR
Voula Kanelis, P. Andrew Chong, and Julie D. Forman-Kay
Part V: CFTR Function
26. Introduction to Part V: Assessment of CFTR Function
Karl Kunzelmann
27. Application of High-Resolution Single-Channel Recording to Functional Studies of Cystic Fibrosis Mutants
Zhiwei Cai, Yoshiro Sohma, Silvia G. Bompadre, David N. Sheppard, and Tzyh-Chang Hwang
28. Electrophysiological, Biochemical, and Bioinformatic Methods for Studying CFTR Channel Gating and Its Regulation
Laszlo Csanady, Paola Vergani, Attila Gulyas-Kovacs, and David C. Gadsby
29. CFTR Regulation by Phosphorylation
Rodrigo Alzamora, J. Darwin King, Jr., and Kenneth R. Hallows
30. How to Measure CFTR-Dependent Bicarbonate Transport: From Single Channels to the Intact Epithelium
Martin J. Hug, Lane L. Clarke, and Michael A. Gray
PRODUCT DETAILS
Publisher: Springer (Humana Press Inc.)
Publication date: May, 2011
Pages: 544
Weight: 1208g
Availability: Available
Subcategories: General Issues, Genetics, Physiology
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