Cystic Fibrosis

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 Includes supplementary material: sn.pub/extras

Inhalt

Introduction to Part I: Overview of Approaches to Study Cystic Fibrosis Pathophysiology.- Imaging CFTR Protein Localization in Cultured Cells and Tissues.- CFTR Regulation of Epithelial Sodium Channel.- Methods for Evaluating Inflammation in Cystic Fibrosis.- Methods for ASL Measurements and Mucus Transport Rates in Cell Cultures.- Measurement of Fluid Secretion from Intact Airway Submucosal Glands.- Measurements of Intracellular Calcium Signals in Polarized Primary Cultures of Normal and Cystic Fibrosis Human Airway Epithelia.- Identification and Quantification of Mucin Expression.- Methods to Classify Bacterial Pathogens in Cystic Fibrosis.- Approaches to Study Differentiation and Repair of Human Airway Epithelial Cells.- Introduction to Part II: Omics in the Biology of Cystic Fibrosis.- Microarray mRNA Expression Profiling to Study Cystic Fibrosis.- Quantitative Differential Proteomics of Cystic Fibrosis Cell Models by SILAC (Stable Isotope Labelling in Cell Culture).- Application of Mass Spectrometry to Study Proteomics and Interactomics in Cystic Fibrosis.- Functional Genomics Assays to Study CFTR Traffic and ENaC Function.- New Lipidomic Approaches in Cystic Fibrosis.- Introduction to Part III: Resources for CFTR Research.- Primary Epithelial Cell Models for Cystic Fibrosis Research.- Comparative Biology of Cystic Fibrosis Animal Models.- CFTR Folding Consortium: Methods Available for Studies of CFTR Folding and Correction.- Evaluation of the Disease Liability of CFTR Variants.