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

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概要 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 ra...nge 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.続きを見る
目次 Introduction to Part I: The Relevance of CF Diagnostic Tools for Measuring Restoration of CFTR Function after Therapeutic Interventions in Human Clinical Trials
High-Throughput Screening of Libraries of Compounds to Identify CFTR Modulators
Repair of CFTR Folding Defects with Correctors that Function as Pharmacological Chaperones
Use of Primary Cultures of Human Bronchial Epithelial Cells Isolated from Cystic Fibrosis Patients for the Pre-Clinical Testing of CFTR Modulators
Design of Gene Therapy Trials in CF Patients
Nasal Potential Difference Measurements to Assess CFTR Ion Channel Activity
Measurement of Ion Transport Function in Rectal Biopsies
Introduction to Part II: RNA Methods to Approach CFTR Expression
Quantification of CFTR Transcripts
Nonsense-Mediated mRNA Decay and Cystic Fibrosis
Approaches to Study CFTR Pre-mRNA Splicing Defects
Impact of MicroRNA in Normal and Pathological Respiratory Epithelia
Genomic Approaches to Studying CFTR Transcriptional Regulation
Introduction to Part III: Biochemical Methods to Study CFTR Protein
Analysis of CFTR Folding and Degradation in Transiently Transfected Cells
In Vitro Methods for CFTR Biogenesis
Analysis of CFTR Interactome in the Macromolecular Complexes
Methods to Monitor Cell Surface Expression and Endocytic Trafficking of CFTR in Polarized Epithelial Cells
Segmental and Subcellular Distribution of CFTR in the Kidney
Endocytic Sorting of CFTR Variants Monitored by Single Cell Fluorescence Ratio Image Analysis (FRIA) in Living Cells
Introduction to Part IV: Biophysical Methods to Approach CFTR Structure
CFTR Three-Dimensional Structure
Molecular Modeling Tools and Approaches for CFTR and Cystic Fibrosis
Biochemical and Biophysical Approaches to Probe CFTR Structure
NMR Spectroscopy to Study the Dynamics and Interactions of CFTR
Introduction to Part V: Assessment of CFTR Function
Application of High-Resolution Single-Channel Recording to Functional Studies of Cystic Fibrosis Mutants
Electrophysiological, Biochemical, and Bioinformatic Methods for Studying CFTR Channel Gating and Its Regulation
CFTR Regulation by Phosphorylation
How to Measure CFTR-Dependent Bicarbonate Transport: From Single Channels to the Intact Epithelium.
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登録日 2020.06.27
更新日 2020.06.28