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Cystic Fibrosis Margarida D. Amaral

Cystic Fibrosis By Margarida D. Amaral

Cystic Fibrosis by Margarida D. Amaral


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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 Summary

Cystic Fibrosis: Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects by Margarida D. Amaral

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.

Cystic Fibrosis Reviews

From the reviews:

Summarize the current complex information on cystic fibrosis (CF) and the innovative new technologies available to basic scientists involved in the study of CF. ... This detailed book is aimed at basic research scientists and academicians working on CF. The protocols would be of use to graduate students and postdoctoral fellows as well. ... technology described would be invaluable to clinical laboratories involved in the diagnosis of CF. This represents a must-have guide for research laboratories working on the functional mechanisms of the CFTR gene. (Luis F. Escobar, Doody's Book Reviews, March, 2012)

Table of 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

Additional information

NLS9781493957873
9781493957873
1493957872
Cystic Fibrosis: Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects by Margarida D. Amaral
New
Paperback
Humana Press Inc.
2016-08-23
528
N/A
Book picture is for illustrative purposes only, actual binding, cover or edition may vary.
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