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Cystic Fibrosis: Diagnosis and Protocols, Volume 1: Approaches to Study and Correct CFTR Defects
Hardcover
Series: Methods in Molecular Biology, Book 741
ISBN13: 9781617791161
Publisher: Springer Nature
Published: May 25 2011
Pages: 528
Weight: 2.55
Height: 1.40 Width: 7.10 Depth: 10.10
Language: English
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
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