Cystic Fibrosis Methods and Protocols by William R. SkachCystic Fibrosis Methods and Protocols by William R. Skach

Cystic Fibrosis Methods and Protocols

EditorWilliam R. Skach

Paperback | November 2, 2011

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Cystic Fibrosis: Methods and Protocols consolidates cutting-edge in vitro, cellular, and whole animal laboratory protocols into an indispensable resource. From electrophysiology and cell biology, to animal models and gene therapy, this comprehensive set of methods provides the step-by-step instructions needed for investigators to incorporate new approaches into their research programs. Specific protocols describe new techniques for diagnosis, in vitro methods for the expression and functional analysis of CFTR, novel biochemical and cellular systems to determine how mutations subvert CFTR function, and in vivo protocols to examine how CFTR dysfunction produce multisystem pathology in both human and animal models.
Title:Cystic Fibrosis Methods and ProtocolsFormat:PaperbackDimensions:648 pagesPublished:November 2, 2011Publisher:Humana PressLanguage:English

The following ISBNs are associated with this title:

ISBN - 10:1617372447

ISBN - 13:9781617372445

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Table of Contents

Part I. Genetics of Cystic FibrosisCFTR Mutation Detection by Multiplex Heteroduplex (mHET) Analysis on MDE GelJulian Zielenski, Isabel Aznarez, Tuncer Onay, John Tzounzouris, Danuta Markiewicz, and Lap-Chee TsuicDNA Microarrays for Pharmacogenomic Analysis of Cystic Fibrosis, Meera SrivastavaOfer Eidelman, and Harvey B. PollardNatural Animal Models of Human Genetic DiseasesJeffrey J. Wine, Michael Dean, and Damjan GlavacPart II. CFTR Structure and Function: Structure, Gating, and RegulationElectrophysiological Approach to Studying CFTRHorst FischerQuantitative Analysis of ATP-Dependent Gating of CFTRAllan Powe, Zhen Zhou, Tzyh-Chang Hwang, and Georg NagelCFTR Regulation by PhosphorylationTang Zhu, Deborah A. R. Hinkson, David Dahan, Alexandra Evagelidis, and John W. HanrahanTransepithelial Measurements of Bicarbonate Secretion in Calu-3 CellsRobert J. BridgesTransepithelial Impedance Analysis of Chloride SecretionAshvani K. Singh, Sangeeta Singh, Daniel C. Devor, Raymond A. Frizzell, Willy van Driessche, and Robert J. BridgesStudies of the Molecular Basis for Cystic Fibrosis Using Purified Reconstituted CFTR ProteinIlana Kogan, Mohabir Ramjeesingh, Canhui Li, and Christine E. BearProbing CFTR Channel Structure and Function Using the Substituted-Cysteine-Accessibility MethodMyles H. AkabasMethods for the Study of Intermolecular and Intramolecular Interactions Regulating CFTR FunctionAnjaparavanda P. NarenFluorescent Indicator Methods to Assay Functional CFTR Expression in CellsAlan S. Verkman and Sujatha JayaramanExpression, Folding, and DegradationImmunolocalization of CFTR in Intact Tissue and Cultured CellsChristopher R. MarinoAnalysis of CFTR Trafficking and Polarization Using Green Fluorescent Protein and Confocal MicroscopyBryan D. Moyer and Bruce A. StantonCFTR Folding and Maturation in CellsMohamed Benharouga, Manu Sharma, and Gergely L. LukacsIsolation of CFTR: Chaperone Complexes by Co-ImmunoprecipitationGeoffrey C. Meacham and Douglas M. CyrCFTR Expression and ER-Associated Degradation in YeastYimao Zhang, Susan Michaelis, and Jeffrey L. BrodskyManipulating the Folding Pathway of -F508 CFTR Using Chemical ChaperonesMarybeth Howard and William J. WelchCFTR Degradation and AggregationMichael J. Corboy, Philip J. Thomas, and W. Christian WigleyIn Vitro Reconstitution of CFTR Biogenesis and DegradationJon Oberdorf and William R. SkachIn Vitro CFTR Folding AssaysRhesa D. Stidham, W. Christian Wigley, and Philip J. ThomasAnalysis of CFTR Endocytosis by Cell Surface BiotinylationKelly Weixel and Neil A. BradburyRegulatory ComplexesCFTR Regulation of ENaCScott H. Donaldson, Elaine G. Poligone, and M. Jackson StuttsYeast Two-Hybrid Identification and Analysis of Protein Interactions with CFTRViswanathan Raghuram, Kenneth R. Hallows, and J. Kevin FoskettBiochemical Assays for Studying Indirect Interactions Between CFTR and the CytoskeletonPeter J. Mohler, Patricia L. Kultgen, M. Jackson Stutts, and Sharon L. MilgramCFTR-Associated ATP Transport and ReleaseMarie E. EganPart III. Pathophysiology of Cystic FibrosisInflammatory Mediators in CF Patients,Jay B. Hilliard, Michael W. Konstan, and Pamela B. DavisBacterial Colonization and Infection in the CF LungScott D. Sagel, Elaine B. Dowell, and Frank J. AccursoAntimicrobial Peptides and Proteins in the CF Airway,Alexander M. Cole and Tomas GanzBacterial-Epithelial InteractionsRuth Bryan and Alice PrinceThin-Film Measurements of Airway Surface Liquid Volume/Composition and Mucus Transport Rates In VitroRobert Tarran and Richard C. BoucherAnimal Models of Cystic FibrosisMurine Models of CF Airway Infection and InflammationJames F. Chmiel, Michael W. Konstan, and Melvin BergerAnalysis of Lipid Abnormalities in CF MiceSteven D. Freedman, Paola G. Blanco, Julie C. Shea, and Juan G. AlvarezBioelectric Measurement of CFTR Function in MiceBarbara R. GrubbXenograft Model of the CF AirwayMohammed Filali, Yulong Zhang, Teresa C. Ritchie, and John F. EngelhardtDevelopment of Conditionally Immortalized Epithelial Cell Lines from CF and Non-CF MiceCalvin U. CottonTechnical Approaches to Analyze the In Vivo Ion Composition of Airway Surface LiquidJean-Marie Zahm, Sonia Baconnais, Gérard Balossier, and Edith PuchellePart IV. Novel Therapeutic Approaches for Cystic FibrosisDesign of Gene Therapy Clinical Trials in CF PatientsKimberly V. Curlee and Eric J. SorscherFormulation of Synthetic Vectors for Cystic Fibrosis Gene TherapyJohn Marshall and Seng H. ChengAdeno-Associated Viral Vectors for CF Gene TherapyTerence R. Flotte, Isabel Virella-Lowell, and Kye A. Chesnut

Editorial Reviews

"The coverage of methodologies is extremely broad and will be useful for those wanting to find out more about some of the less routine techniques used in the analysis of the CFTR protein/chloride channel. The style of protocol-based chapters enables in manuscripts due to considerations of space or potential repetition of previously published techniques. As such many of the chapters are valuable." -Molecular Biotechnology"Undoubtedly this book will be regarded as essential reading for scientists in the field. It provides details of research methodology, which are rarely published in peer reviewed journals. It will also enable researchers to identify subtle differences in techniques in their own laboratories from those described in these chapters. As such, it will be a very important book for all those who conduct laboratory-based research into cystic fibrosis." -Journal of Paediatrics and Child Health