Cystic Fibrosis Methods and Protocols

Genetics of Cystic Fibrosis.- CFTR Mutation Detection by Multiplex Heteroduplex (mHET) Analysis on MDE Gel.- cDNA Microarrays for Pharmacogenomic Analysis of Cystic Fibrosis.- Natural Animal Models of Human Genetic Diseases.- CFTR structure and Function:.- Electrophysiological Approach to Studying CFTR.- Quantitative Analysis of ATP-Dependent Gating of CFTR.- CFTR Regulation by Phosphorylation.- Transepithelial Measurements of Bicarbonate Secretion in Calu-3 Cells.- Transepithelial Impedance Analysis of Chloride Secretion.- Studies of the Molecular Basis for Cystic Fibrosis Using Purified Reconstituted CFTR Protein.- Probing CFTR Channel Structure and Function Using the Substituted-Cysteine-Accessibility Method.- Methods for the Study of Intermolecular and Intramolecular Interactions Regulating CFTR Function.- Fluorescent Indicator Methods to Assay Functional CFTR Expression in Cells.- Immunolocalization of CFTR in Intact Tissue and Cultured Cells.- Analysis of CFTR Trafficking and Polarization Using Green Fluorescent Protein and Confocal Microscopy.- CFTR Folding and Maturation in Cells.- Isolation of CFTR.- CFTR Expression and ER-Associated Degradation in Yeast.- Manipulating the Folding Pathway of ?F508 CFTR Using Chemical Chaperones.- CFTR Degradation and Aggregation.- In Vitro Reconstitution of CFTR Biogenesis and Degradation.- In Vitro CFTR Folding Assays.- Analysis of CFTR Endocytosis by Cell Surface Biotinylation.- CFTR Regulation of ENaC.- Yeast Two-Hybrid Identification and Analysis of Protein Interactions with CFTR.- Biochemical Assays for Studying Indirect Interactions Between CFTR and the Cytoskeleton.- CFTR-Associated ATP Transport and Release.- Pathophysiology of Cystic Fibrosis.- Inflammatory Mediators in CF Patients.- Bacterial Colonization andInfection in the CF Lung.- Antimicrobial Peptides and Proteins in the CF Airway.- Bacterial-Epithelial Interactions.- Thin-Film Measurements of Airway Surface Liquid Volume/Composition and Mucus Transport Rates In Vitro.- Murine Models of CF Airway Infection and Inflammation.- Analysis of Lipid Abnormalities in CF Mice.- Bioelectric Measurement of CFTR Function in Mice.- Xenograft Model of the CF Airway.- Development of Conditionally Immortalized Epithelial Cell Lines from CF and Non-CF Mice.- Technical Approaches to Analyze the In Vivo Ion Composition of Airway Surface Liquid.- Novel Therapeutic Approaches for Cystic Fibrosis.- Design of Gene Therapy Clinical Trials in CF Patients.- Formulation of Synthetic Vectors for Cystic Fibrosis Gene Therapy.- Adeno-Associated Viral Vectors for CF Gene Therapy.