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Cystic Fibrosis

  • Kartonierter Einband
  • 400 Seiten
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This volume details the cystic fibrosis transmembrane conductance regulator and its expression, biogenesis, structure and function... Weiterlesen
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This volume details the cystic fibrosis transmembrane conductance regulator and its expression, biogenesis, structure and function in terms of the defects causing cystic fibrosis. It features readily reproducible laboratory protocols and troubleshooting tips.

Despite the many milestones in cystic fibrosis (CF) research, progress toward 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 II: Methods and Resources to Understand Cystic Fibrosis focuses on pathophysiology, Omics approaches, and a variety of key resources recently made available for CF research. 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.

Features cutting-edge protocols from the highly supportive CF scientific community

Contains expert tips and key implementation advice

Focuses on cystic fibrosis pathophysiology and Omics studies with an emphasis on practical approaches


Part I: Pathophysiology of Cystic Fibrosis

1. Introduction to Part I: Overview of Approaches to Study Cystic Fibrosis Pathophysiology

Mark T. Clunes and Richard C. Boucher

2. Imaging CFTR Protein Localization in Cultured Cells and Tissues

Silvia M. Kreda and Martina Gentzsch

3. CFTR Regulation of Epithelial Sodium Channel

Yawar J. Qadri, Estelle Cormet-Boyaka, Dale J. Benos, and Bakhrom K. Berdiev

4. Methods for Evaluating Inflammation in Cystic Fibrosis

Assem G. Ziady and Pamela B. Davis

5. Methods for ASL Measurements and Mucus Transport Rates in Cell Cultures

Erin N. Worthington and Robert Tarran

6. Measurement of Fluid Secretion from Intact Airway Submucosal Glands

Jeffrey J. Wine, Nam Soo Joo, Jae Young Choi, Hyungju Cho, Mauri E. Krouse, Jin V. Wu, Monal Khansaheb, Toshiya Irokawa, Juan Ianowski, John W. Hanrahan, Alan W. Cuthbert, and Kim V. Tran

7. Measurements of Intracellular Calcium Signals in Polarized Primary Cultures of Normal and Cystic Fibrosis Human Airway Epithelia

Carla M.P. Ribeiro

8. Identification and Quantification of Mucin Expression

Kristina A. Thomsson and Gunnar C. Hansson

9. Methods to Classify Bacterial Pathogens in Cystic Fibrosis

Thomas Bjarnsholt, Xiaohui Chen Nielsen, Ulla Johansen, Lena Nørgaard, and Niels Høiby

10. Approaches to Study Differentiation and Repair of Human Airway Epithelial Cells

Sophie Crespin, Marc Bacchetta, Song Huang, Tecla Dudez, Ludovic Wiszniewski, and Marc Chanson

Part II: Omic Approaches to Study Cystic Fibrosis

11. Introduction to Part II: Omics in the Biology of Cystic Fibrosis

William E. Balch

12. Microarray mRNA Expression Profiling to Study Cystic Fibrosis

Shyam Ramachandran, Luka A. Clarke, Todd E. Scheetz, Margarida D. Amaral, and Paul B. McCray, Jr.

13. Quantitative Differential Proteomics of Cystic Fibrosis Cell Models by SILAC (Stable Isotope Labelling in Cell Culture)

Ida Chiara Guerrera, Mario Ollero, Diane-Lore Vieu, and Aleksander Edelman

14. Application of Mass Spectrometry to Study Proteomics and Interactomics in Cystic Fibrosis

William E. Balch and John R. Yates III

15. Functional Genomics Assays to Study CFTR Traffic and ENaC Function

Joana Almaça, Shehrazade Dahimène, Nicole Appel, Christian Conrad, Karl Kunzelmann, Rainer Pepperkok, and Margarida D. Amaral

16. New Lipidomic Approaches in Cystic Fibrosis

Mario Ollero, Ida Chiara Guerrera, Giuseppe Astarita, Daniele Piomelli, and Aleksander Edelman

Part III: Resources

17. Introduction to Part III: Resources for CFTR Research

Margarida D. Amaral

18. Primary Epithelial Cell Models for Cystic Fibrosis Research

Scott H. Randell, M. Leslie Fulcher, Wanda O'Neal, and John C. Olsen

19. Comparative Biology of Cystic Fibrosis Animal Models

John T. Fisher, Yulong Zhang, and John F. Engelhardt

20. CFTR Folding Consortium: Methods Available for Studies of CFTR Folding and Correction

Kathryn W. Peters, Tsukasa Okiyoneda, William E. Balch, Ineke Braakman, Jeffrey L. Brodsky, William B. Guggino, Christopher M. Penland, Harvey B. Pollard, Eric J. Sorscher, William R. Skach, Philip J. Thomas, Gergely L. Lukacs, and Raymond A. Frizzell

21. Evaluation of the Disease Liability of CFTR Variants

Patrick R. Sosnay, Carlo Castellani, Mary Corey, Ruslan Dorfman, Julian Zielenski, Rachel Karchin, Christopher M. Penland, and Garry R. Cutting


Titel: Cystic Fibrosis
Untertitel: Diagnosis and Protocols, Volume II: Methods and Resources to Understand Cystic Fibrosis
EAN: 9781493962822
ISBN: 1493962825
Format: Kartonierter Einband
Herausgeber: Humana Press
Genre: Medizin
Anzahl Seiten: 400
Gewicht: 750g
Größe: H254mm x B178mm x T21mm
Jahr: 2016
Auflage: Softcover reprint of the original 1st ed. 2011

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