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Cystic Fibrosis

  • Kartonierter Einband
  • 544 Seiten
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This volume details the cystic fibrosis transmembrane conductance regulator and its expression, biogenesis, structure and function... Weiterlesen
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Beschreibung

This volume details the cystic fibrosis transmembrane conductance regulator and its expression, biogenesis, structure and function in terms of the defects causing cystic fibrosis. It features readily reproducible laboratory protocols and troubleshooting tips.


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.

Features cutting-edge protocols from the highly supportive CF scientific community

Contains expert tips and key implementation advice

Focuses on Cystic fibrosis transmembrane conductance regulator defects with an emphasis on practical approaches



Inhalt
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 François 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 Barrière, 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, André 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 André 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 László Csanády, Paola Vergani, Attila Gulyás-Kovács, 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

Produktinformationen

Titel: Cystic Fibrosis
Untertitel: Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects
Editor:
EAN: 9781493957873
ISBN: 1493957872
Format: Kartonierter Einband
Herausgeber: Humana Press
Genre: Medizin
Anzahl Seiten: 544
Gewicht: 1011g
Größe: H254mm x B178mm x T29mm
Jahr: 2016
Untertitel: Englisch
Auflage: Softcover reprint of the original 1st ed. 2011

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