Cystic fibrosis: diagnosis and protocols 1 Approaches to study and correct CFTR defects
Gespeichert in:
Weitere Verfasser: | |
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Format: | Buch |
Sprache: | English |
Veröffentlicht: |
New York [u.a.]
Humana Press [u.a.]
2011
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Schriftenreihe: | Methods in molecular biology
741 |
Online-Zugang: | Inhaltsverzeichnis |
Beschreibung: | XVI, 528 S. Ill., graph. Darst. |
ISBN: | 9781617791161 |
Internformat
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245 | 1 | 0 | |a Cystic fibrosis |b diagnosis and protocols |n 1 |p Approaches to study and correct CFTR defects |c ed. by Margarida D. Amaral ; Karl Kunzelmann |
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Datensatz im Suchindex
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adam_text | Titel: Bd. 1. Cystic fibrosis. Approaches to study and correct CFTR defects
Autor:
Jahr: 2011
Contents
Foreword......................................... v
Preface . ,........................................ vit
Contributors...................................... . xiii
Section I: Strategies to Correct the Basic Defect in CF and Assess
Efficacy in Human Clinical Trials
1. Introduction to Section I: The Relevance of CF Diagnostic Tools
for Measuring Restoration of CFTR Function After Therapeutic
Interventions in Human Clinical Trials....................... 3
Kris De Boeck and Melissa Ashlock
2. High-Throughput Screening of Libraries of Compounds to Identify
CFTR Modulators................................. 13
Nicoletta Pedemontey Olga Zejjarru-Moran, and Luis J. V. Galietta
3. Repair of CFTR Folding Defects with Correctors that Function as
Pharmacological Chaperones ............................ 23
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 . . 39
Timothy Neuberger, Bill Burton, Heather Clark, and Fredrick Van Goor
5. Design ofGene Therapy Trials in CF Patients................... 55
Jame C. Davies and Eric W.F.W Alton
6. Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity . . 69
Steven M, Rime, John Paul Clancy, and Michael Wilschamki
7. Measurement of Ion Transport Function in Rectal Biopsies ............ 87
Marti» J. Hug, Nico Derichs, Inez Bronsveld, and Jean Paul Clancy
Section II: RNA Methods to Approach CFTR Expression
8. Introduction to Section II: RNA Methods to Approach CFTR Expression .... Ill
Ann Harris
9. Quantification of CFTR Transcripts........................115
Anabela S. Ramalho, LukaA. Clarke, and Margarida D. Amaral
10. Nonsense-Mediated mRNA Decay and Cystic Fibrosis ............... 137
LiatLimde and Batsheva Kerem
x Contente
11. Approaches to Study CFTR Pre-mRNA Splicing Defects .............155
Elisa Goina, Eugenia Femandez-Alanis, and Franco Pagani
12. Impact of MicroRNA in Normal and Pathological Respiratory Epithelia . ..... 171
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 .......193
Christopher J. Ott and Ann Harris
Section III: CFTR Protein Biogenesis, Folding, Degradation, and Traffic
14. Introduction to Section III: Biochemical Methods to Study CFTR Protein .... 213
Margarida D. Amaral and Gergety L. Lukacs
15. Analysis of CFTR Folding and Degradation in Transiently Transfected Cells ... 219
Diane E. Grove, Meredith F.N. Rosser, Richard L. Watkins, and Douglas
M. Cyr
16. In Vitro Methods for CFTR Biogenesis......................233
Toshihiro Mafáumura, LeeAmn Roomy, and William R. Skach
17. Analysis of CFTR Interactome in the Macromolecular Complexes......... 255
Chunying Li and Anjaparavanda P. Naren
18. Methods to Monitor Cell Surface Expression and Endocytic Trafficking
of CFTR in Polarized Epithelial Cells.......................271
Jennifer M. Bamberger, William B. Guggino, and Bruce A. Stanton
19. Segmental and Subcellular Distribution of CFTR in the Kidney..........285
Francois Jouret, Pierre J. Courtoy, and Olivier Devuyst
20. Endocytic Sorting of CFTR Variants Monitored by Single-Cell
Fluorescence Ratiometric Image Analysis (FRIA) in Living Cells..........301
Hervé Barrière, Pirjo Apaja, Trnkasa Okiyoneda,
and Gergely L, Lukacs
Section IV: CFTR Structure
21. Introduction to Section IV: Biophysical Methods to Approach CFTR Structure . . 321
Juan L. Mendoza, André Schmidt, and Philip J. Thomas
22. CFTR Three-Dimensional Structure........................329
Robert C. Ford, James Birtley, Mark F. Rosenberg, and Liang Zhang
23. Molecular Modeling Tools and Approaches for CFTR and Cystic Fibrosis.....347
Adrian W.R, Serohißs, Patrick H. Tbibodeau,
and Nikolay V. Dokholyan
24. Biochemical and Biophysical Approaches to Probe CFTR Structure ........ 365
André Schmidt, Juan L. Mendoza, and Philip J. Thomas
Contents xi
25. NMR Spectroscopy to Study the Dynamics and Interactions of CFTR.......377
Voula Kanelis, P. Andrew Chong, and Julie D. Forman-Kay
Section V: CFTR Function
26. Introduction to Section V: Assessment of CFTR Function............. 407
Karl Kunzelmann
27. Application of High-Resolution Single-Channel Recording to Functional
Studies of Cystic Fibrosis Mutants........................ . 419
Zhiwei Cai, Yoshiro Sohma, Silvia G. Bompadre,
David N. Sheppard, and Tzyb-Chang Hwang
28. Electrophysiological, Biochemical, and Bioinformatic Methods for
Studying CFTR Channel Gating and Its Regulation................ 443
Ldszló Csanédy, Paola Vergani, Attila Gulyés-Kovács, and David C. Gadsby
29. CFTR Regulation by Phosphorylation...................... . 471
Rodrigo Ahamora, J Darwin King, and Kenneth R. Hallows
30. How to Measure CFTR-Dependent Bicarbonate Transport: From Single
Channels to the Intact Epithelium......................... 489
Martin J. Hug, LaneL. Clarke, and Michael A. Gray
Index...........................................511
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spelling | Cystic fibrosis diagnosis and protocols 1 Approaches to study and correct CFTR defects ed. by Margarida D. Amaral ; Karl Kunzelmann New York [u.a.] Humana Press [u.a.] 2011 XVI, 528 S. Ill., graph. Darst. txt rdacontent n rdamedia nc rdacarrier Methods in molecular biology 741 Methods in molecular biology ... Springer protocols Amaral, Margarida D. edt (DE-604)BV039146574 1 Methods in molecular biology 741 (DE-604)BV035362695 741 HBZ Datenaustausch application/pdf http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=024164480&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA Inhaltsverzeichnis |
spellingShingle | Cystic fibrosis diagnosis and protocols Methods in molecular biology |
title | Cystic fibrosis diagnosis and protocols |
title_auth | Cystic fibrosis diagnosis and protocols |
title_exact_search | Cystic fibrosis diagnosis and protocols |
title_full | Cystic fibrosis diagnosis and protocols 1 Approaches to study and correct CFTR defects ed. by Margarida D. Amaral ; Karl Kunzelmann |
title_fullStr | Cystic fibrosis diagnosis and protocols 1 Approaches to study and correct CFTR defects ed. by Margarida D. Amaral ; Karl Kunzelmann |
title_full_unstemmed | Cystic fibrosis diagnosis and protocols 1 Approaches to study and correct CFTR defects ed. by Margarida D. Amaral ; Karl Kunzelmann |
title_short | Cystic fibrosis |
title_sort | cystic fibrosis diagnosis and protocols approaches to study and correct cftr defects |
title_sub | diagnosis and protocols |
url | http://bvbr.bib-bvb.de:8991/F?func=service&doc_library=BVB01&local_base=BVB01&doc_number=024164480&sequence=000002&line_number=0001&func_code=DB_RECORDS&service_type=MEDIA |
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