Cystic Fibrosis : Diagnosis and Protocols, Volume I: Approaches to Study and Correct CFTR Defects / edited by Margarida D. Amaral, Karl Kunzelmann.

Format:

Book

Contributor:

Amaral, Margarida D., editor.

Kunzelmann, Karl, editor.

SpringerLink (Online service)

Series:

Methods in Molecular Biology, Methods and Protocols, 1064-3745 ; 741.

Springer Protocols (Springer-12345)

Methods in Molecular Biology, Methods and Protocols, 1064-3745 ; 741

Language:

English

Subjects (All):

Medicine.

Human genetics.

Diagnosis, Laboratory.

Molecular biology.

Biomedicine.

Molecular Medicine.

Human Genetics.

Laboratory Medicine.

Local Subjects:

Biomedicine.

Molecular Medicine.

Human Genetics.

Laboratory Medicine.

Physical Description:

1 online resource (XVI, 528 pages) : 76 illustrations, 4 illustrations in color.

Contained In:

Springer eBooks

Place of Publication:

Totowa, NJ : Humana Press, 2011.

System Details:

text file PDF

Summary:

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.

Contents:

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.

Other Format:

Printed edition:

ISBN:

9781617791178

Access Restriction:

Restricted for use by site license.