Analysis of nasal potential in murine cystic fibrosis models

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• 作者：Mé ; lanie Faria da Cunha^a ; ¹ ; Juliette Simonin^a ; ¹ ; Ali Sassi^a ; Romain Freund^b ; Auré ; lie Hatton^a ; Charles-Henry Cottart^a ; Nadia Elganfoud^a ; Rachid Zoubairi^a ; Corina Dragu^a ; Jean Philippe Jais^b ; Alexandre Hinzpeter^a ; Aleksander Edelman^a ; Isabelle Sermet-Gaudelus^a ; ^{isabelle.sermet@aphp.fr" class="auth_mail" title="E-mail the corresponding author}
• 关键词：ABCC7 ; ATP binding cassette subfamily C member 7 ; ATP ; Adenosine triphosphate ; BALF ; Bronchoalveolar lavage fluid ; CaCC ; Ca2+ activated chloride channel ; cAMP ; 3&prime ; 5&prime ; -cyclic adenosine monophosphate ; CF ; Cystic fibrosis ; CFTR ; Cystic fibrosis transmembrane conductance regulator ; ClC ; Chloride channel family ; CLCN2 ; Chloride channel protein 2 ; CNG ; Cyclic nucleotide gated ; DIDS ; Disodium 4 ; 4&prime ; -diisothiocyanatostilbene-2 ; 2&prime ; -disul​fonate ; DPC ; Diphenylamine-2-carboxylic acid ; E
• 刊名：International Journal of Biochemistry and Cell Biology
• 出版年：2016
• 出版时间：November 2016
• 年：2016
• 卷：80
• 期：Complete
• 页码：87-97
• 全文大小：1173 K

文摘

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.