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Cystic Fibrosis: An autosomal recessive genetic disease of the Exocrine glands. It is caused by mutations in the gene encoding the Cystic fibrosis transmembrane conductance regulator expressed in several organs including the Lung, the Pancreas, the Biliary system, and the Sweat glands. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in Airway obstruction; chronic Respiratory infections; Pancreatic insufficiency; maldigestion; salt depletion; and Heat prostration.

Standardized Measurement of Nasal Membrane Transepithelial Potential Difference (NPD)

1Department of Medicine and the Gregory Fleming James Cystic Fibrosis Center, University of Alabama at Birmingham, 2Department of Pulmonology and Tuberculosis, University Medical Center Utrecht, 3Center for Experimental Medicine, Queens University, Northern Ireland, 4Hadassah Hebrew University Medical Center, Jerusalem, 5Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata, 6Service de Pneumologie et Allergologie Pédiatriques and Center de Ressources et de Compétence de la Mucoviscidose, Hôpital Necker Enfants Malades, 7INSERM U 1151, Institut Necker Enfants Malades

JoVE 57006


 Medicine

Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients

1Foundation Hubrecht Organoid Technology, 2Department of Pediatric Pulmonology, Regenerative Medicine Centre Utrecht, Wilhelmina Children's Hospital, University Medical Centre Utrecht, 3Department of Stem Cells and Cancer, Walter and Eliza Hall Institute of Medical Research, 4Hubrecht Institute for Developmental Biology and Stem Cell Research, University Medical Centre Utrecht

JoVE 55159


 Medicine

A Novel Surgical Approach for Intratracheal Administration of Bioactive Agents in a Fetal Mouse Model

1Molecular Virology and Gene Therapy, KU Leuven, 2Department of Woman and Child, KU Leuven, 3Neurobiology and Gene Therapy, KU Leuven, 4Division of Nuclear Medicine, KU Leuven, 5Biomedical NMR Unit/ MoSAIC, KU Leuven

JoVE 4219


 Medicine

Genetic Engineering of Primary Mouse Intestinal Organoids Using Magnetic Nanoparticle Transduction Viral Vectors for Frozen Sectioning

1Department of Medicine, Division of Hematology, Johns Hopkins University School of Medicine, 2Department of Cell Biology, Johns Hopkins University School of Medicine, 3Department of Oncology, Johns Hopkins University School of Medicine, 4Department of Pathology, Johns Hopkins University School of Medicine, 5Institute for Cell Engineering, Johns Hopkins University School of Medicine

Video Coming Soon

JoVE 57040


 JoVE In-Press

Replication of the Ordered, Nonredundant Library of Pseudomonas aeruginosa strain PA14 Transposon Insertion Mutants

1Department of Pediatrics, Mucosal Immunology and Biology Research Center, Massachusetts General Hospital, 2Department of Molecular Biology, Massachusetts General Hospital, 3Department of Genetics, Harvard Medical School, 4Department of Pediatrics, Harvard Medical School

JoVE 57298


 Immunology and Infection

Deciphering and Imaging Pathogenesis and Cording of Mycobacterium abscessus in Zebrafish Embryos

1Dynamique des Interactions Membranaires Normales et Pathologiques, CNRS, UMR 535, Université Montpellier, 2Centre d'études d'agents Pathogènes et Biotechnologies pour la Santé, CNRS, FRE 3689, Université Montpellier, 3Unité de Formation et de Recherche des Sciences de la Santé, EA3647-EPIM, Université Versailles St Quentin

JoVE 53130


 Immunology and Infection

Live Cell Analysis of Shear Stress on Pseudomonas aeruginosa Using an Automated Medium-Throughput Microfluidic System

1Department of Chemistry, Doane University, 2Department of Biology, Doane University, 3Department of Pathology and Microbiology, University of Nebraska Medical Center, 4Department of Physics and Engineering, Doane University

Video Coming Soon

JoVE 58926


 JoVE In-Press

Generation of Cationic Nanoliposomes for the Efficient Delivery of In Vitro Transcribed Messenger RNA

1Department of Thoracic and Cardiovascular Surgery, Clinical Research Laboratory, University Medical Center, 2Atherothrombosis and Vascular Biology, Baker Heart & Diabetes Institute, 3Department of Medicine, Monash University

Video Coming Soon

JoVE 58444


 JoVE In-Press

Label-free Neutrophil Enrichment from Patient-derived Airway Secretion Using Closed-loop Inertial Microfluidics

1Research Laboratory of Electronics, Massachusetts Institute of Technology, 2Department of Electrical Engineering and Computer Science, Massachusetts Institute of Technology, 3Department of Medicine, University of Pittsburgh, 4Department of Biological Engineering, Massachusetts Institute of Technology, 5Vascular Medicine Institute, University of Pittsburgh

JoVE 57673


 Immunology and Infection

A Protein Microarray Assay for Serological Determination of Antigen-specific Antibody Responses Following Clostridium difficile Infection

1Breast Surgery Group, Division of Medical Sciences and Graduate Entry Medicine, School of Medicine, Queen's Medical Centre, University of Nottingham, 2Medical Microbiology and Immunology Department, Faculty of Medicine, Mansoura University, 3Nottingham Digestive Diseases Centre and NIHR Nottingham Digestive Diseases Biomedical Research Centre, School of Medicine, Queen's Medical Centre, University of Nottingham

JoVE 57399


 Immunology and Infection

An IL-8 Transiently Transgenized Mouse Model for the In Vivo Long-term Monitoring of Inflammatory Responses

1Department of Medicine, General Pathology Division, Cystic Fibrosis Translational Research Laboratory "D. Lissandrini", University of Verona, 2Corporate Preclinical R&D, Chiesi Farmaceutici S.p.A., 3Department of Diagnostic and Public Health, Microbiology Division, University of Verona, 4Dipartimento di Scienze Medico-Veterinarie, University of Parma, 5Department of Biomedical Biotechnological and Translational Sciences, University of Parma, 6Department of Computer Science, University of Verona, 7Department of Neurological, Biomedical and Movement Sciences, University of Verona, 8Cystic Fibrosis Regional Center (CFC), AOUI Verona

JoVE 55499


 Immunology and Infection

The α-test: Rapid Cell-free CD4 Enumeration Using Whole Saliva

1Department of Medicine, Weill Cornell Medical College, 2Department of Oral Biology, University of Missouri-Kansas City-School of Dentistry, 3Department of Pharmacology and Toxicology, University of Missouri Kansas City- School of Pharmacy, 4Regional Hospital, Bamenda, NWP, Cameroon, 5Mezam Polyclinic HIV/AIDS Treatment Center, Cameroon, 6Institute for Human Genetics and Biochemistry

JoVE 3999


 Medicine

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