Cystic Fibrosis

Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects

  • Margarida D. Amaral
  • Karl Kunzelmann

Part of the Methods in Molecular Biology book series (MIMB, volume 741)

Table of contents

  1. Front Matter
    Pages i-xvi
  2. Strategies to Correct the Basic Defect in CF and Assess Efficacy in Human Clinical Trials

    1. Front Matter
      Pages 1-1
    2. Nicoletta Pedemonte, Olga Zegarra-Moran, Luis J.V. Galietta
      Pages 13-21
    3. Jane C. Davies, Eric W.F.W. Alton
      Pages 55-68
    4. Steven M. Rowe, John Paul Clancy, Michael Wilschanski
      Pages 69-86
    5. Martin J. Hug, Nico Derichs, Inez Bronsveld, Jean Paul Clancy
      Pages 87-107
  3. RNA Methods to Approach CFTR Expression

    1. Front Matter
      Pages 109-109
    2. Anabela S. Ramalho, Luka A. Clarke, Margarida D. Amaral
      Pages 115-135
    3. Liat Linde, Batsheva Kerem
      Pages 137-154
    4. Elisa Goina, Eugenio Fernandez-Alanis, Franco Pagani
      Pages 155-169
    5. Lisa Giovannini-Chami, Nathalie Grandvaux, Laure-Emmanuelle Zaragosi, Karine Robbe-Sermesant, Brice Marcet, Bruno Cardinaud et al.
      Pages 171-191
    6. Christopher J. Ott, Ann Harris
      Pages 193-209
  4. CFTR Protein Biogenesis, Folding, Degradation, and Traffic

    1. Front Matter
      Pages 211-211
    2. Margarida D. Amaral, Gergely L. Lukacs
      Pages 213-218
    3. Diane E. Grove, Meredith F.N. Rosser, Richard L. Watkins, Douglas M. Cyr
      Pages 219-232
    4. Yoshihiro Matsumura, LeeAnn Rooney, William R. Skach
      Pages 233-253
    5. Chunying Li, Anjaparavanda P. Naren
      Pages 255-270
    6. Jennifer M. Bomberger, William B. Guggino, Bruce A. Stanton
      Pages 271-283
    7. François Jouret, Pierre J. Courtoy, Olivier Devuyst
      Pages 285-299
    8. Herve Barrière, Pirjo Apaja, Tsukasa Okiyoneda, Gergely L. Lukacs
      Pages 301-317
  5. CFTR Structure

    1. Front Matter
      Pages 319-319
    2. Juan L. Mendoza, André Schmidt, Philip J. Thomas
      Pages 321-327
    3. Robert C. Ford, James Birtley, Mark F. Rosenberg, Liang Zhang
      Pages 329-346
    4. Adrian W.R. Serohijos, Patrick H. Thibodeau, Nikolay V. Dokholyan
      Pages 347-363
    5. André Schmidt, Juan L. Mendoza, Philip J. Thomas
      Pages 365-376
    6. Voula Kanelis, P. Andrew Chong, Julie D. Forman-Kay
      Pages 377-403
  6. CFTR Function

    1. Front Matter
      Pages 405-405
    2. Zhiwei Cai, Yoshiro Sohma, Silvia G. Bompadre, David N. Sheppard, Tzyh-Chang Hwang
      Pages 419-441
    3. László Csanády, Paola Vergani, Attila Gulyás-Kovács, David C. Gadsby
      Pages 443-469
    4. Rodrigo Alzamora, J Darwin King Jr, Kenneth R. Hallows
      Pages 471-488
  7. Back Matter
    Pages 511-528

About this book


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.


CF CFTR expression Human clinical trials Protein biogenesis and folding RNA methods Structure and function Transmembrane conductance regulator

Editors and affiliations

  • Margarida D. Amaral
    • 1
  • Karl Kunzelmann
    • 2
  1. 1.Faculty of Sciences, Centre for Biodiversity & FunctionalUniversity of LisboaLisboaPortugal
  2. 2., Department of PhysiologyUniversity of RegensburgRegensburgGermany

Bibliographic information

  • DOI
  • Copyright Information Springer Science+Business Media, LLC 2011
  • Publisher Name Humana Press
  • eBook Packages Springer Protocols
  • Print ISBN 978-1-61779-116-1
  • Online ISBN 978-1-61779-117-8
  • Series Print ISSN 1064-3745
  • Series Online ISSN 1940-6029
  • Buy this book on publisher's site
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