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  • © 2011

Cystic Fibrosis

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

  • 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
  • Includes supplementary material: sn.pub/extras

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

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Softcover Book USD 119.99
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Table of contents (30 protocols)

  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. High-Throughput Screening of Libraries of Compounds to Identify CFTR Modulators

      • Nicoletta Pedemonte, Olga Zegarra-Moran, Luis J.V. Galietta
      Pages 13-21
    3. Design of Gene Therapy Trials in CF Patients

      • Jane C. Davies, Eric W.F.W. Alton
      Pages 55-68
    4. Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity

      • Steven M. Rowe, John Paul Clancy, Michael Wilschanski
      Pages 69-86
    5. Measurement of Ion Transport Function in Rectal Biopsies

      • 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. Quantification of CFTR Transcripts

      • Anabela S. Ramalho, Luka A. Clarke, Margarida D. Amaral
      Pages 115-135
    3. Nonsense-Mediated mRNA Decay and Cystic Fibrosis

      • Liat Linde, Batsheva Kerem
      Pages 137-154
    4. Approaches to Study CFTR Pre-mRNA Splicing Defects

      • Elisa Goina, Eugenio Fernandez-Alanis, Franco Pagani
      Pages 155-169
    5. Impact of MicroRNA in Normal and Pathological Respiratory Epithelia

      • Lisa Giovannini-Chami, Nathalie Grandvaux, Laure-Emmanuelle Zaragosi, Karine Robbe-Sermesant, Brice Marcet, Bruno Cardinaud et al.
      Pages 171-191
    6. Genomic Approaches to Studying CFTR Transcriptional Regulation

      • Christopher J. Ott, Ann Harris
      Pages 193-209
  4. CFTR Protein Biogenesis, Folding, Degradation, and Traffic

    1. Front Matter

      Pages 211-211
    2. Introduction to Section III: Biochemical Methods to Study CFTR Protein

      • Margarida D. Amaral, Gergely L. Lukacs
      Pages 213-218
    3. Analysis of CFTR Folding and Degradation in Transiently Transfected Cells

      • Diane E. Grove, Meredith F.N. Rosser, Richard L. Watkins, Douglas M. Cyr
      Pages 219-232
    4. In Vitro Methods for CFTR Biogenesis

      • Yoshihiro Matsumura, LeeAnn Rooney, William R. Skach
      Pages 233-253

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.

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)

Editors and Affiliations

  • Faculty of Sciences, Centre for Biodiversity & Functional, University of Lisboa, Lisboa, Portugal

    Margarida D. Amaral

  • , Department of Physiology, University of Regensburg, Regensburg, Germany

    Karl Kunzelmann

Bibliographic Information

  • Book Title: Cystic Fibrosis

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

  • Editors: Margarida D. Amaral, Karl Kunzelmann

  • Series Title: Methods in Molecular Biology

  • DOI: https://doi.org/10.1007/978-1-61779-117-8

  • Publisher: Humana Totowa, NJ

  • eBook Packages: Springer Protocols

  • Copyright Information: Springer Science+Business Media, LLC 2011

  • Hardcover ISBN: 978-1-61779-116-1Published: 25 May 2011

  • Softcover ISBN: 978-1-4939-5787-3Published: 23 August 2016

  • eBook ISBN: 978-1-61779-117-8Published: 19 May 2011

  • Series ISSN: 1064-3745

  • Series E-ISSN: 1940-6029

  • Edition Number: 1

  • Number of Pages: XVI, 528

  • Number of Illustrations: 72 b/w illustrations, 4 illustrations in colour

  • Topics: Molecular Medicine, Human Genetics, Laboratory Medicine

Buy it now

Buying options

eBook USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Other ways to access