title: Analysis of nasal potential in murine cystic fibrosis models.
abstract: The nasal epithelium of the mouse closely mimics the bioelectrical phenotype of the human airways. Ion transport across the nasal epithelium induces a nasal transepithelial potential difference. Its measurement by a relatively non-invasive method adapted from humans allows in vivo longitudinal measurements of CFTR-dependent ionic transport in the murine nasal mucosa. This test offers a useful tool to assess CFTR function in preclinical studies for novel therapeutics modulating CFTR activity. Here we extensively review work done to assess transepithelial transport in the murine respiratory epithelium in the basal state and after administration of CFTR modulators. Factors of variability and discriminative threshold between the CF and the WT mice for different readouts are discussed.
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Mélanie Faria
authors.initials: MF
authors.lastname: da Cunha
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Juliette
authors.initials: J
authors.lastname: Simonin
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Ali
authors.initials: A
authors.lastname: Sassi
authors.affiliation: Unité de Biostatistiques, Hôpital Necker Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France.
authors.forename: Romain
authors.initials: R
authors.lastname: Freund
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Hatton
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Charles-Henry
authors.initials: CH
authors.lastname: Cottart
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Nadia
authors.initials: N
authors.lastname: Elganfoud
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Rachid
authors.initials: R
authors.lastname: Zoubairi
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Corina
authors.initials: C
authors.lastname: Dragu
authors.affiliation: Unité de Biostatistiques, Hôpital Necker Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France.
authors.forename: Jean Philippe
authors.initials: JP
authors.lastname: Jais
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Hinzpeter
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France.
authors.forename: Aleksander
authors.initials: A
authors.lastname: Edelman
authors.affiliation: INSERM U 1151, Institut Necker Enfants Malades, Université Paris Sorbonne, Paris, France. Electronic address: isabelle.sermet@aphp.fr.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Sermet-Gaudelus
mesh_terms: D000818:Animals; D001692:Biological Transport; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D004195:Disease Models, Animal; D004848:Epithelium / Q000378:metabolism / Q000473:pathology; D006801:Humans; D009297:Nasal Mucosa / Q000378:metabolism; D009666:Nose / Q000473:pathology
keywords: CFTR correctors; Cystic fibrosis; Ionic transepithelial transport; Mouse; Nasal potential difference