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Rectal potential difference and the functional expression of CFTR in the gastrointestinal epithelia in cystic fibrosis mouse models.
Pediatr Res 2008; 63(1):73-8PR

Abstract

Cystic fibrosis (CF) is an autosomal recessive disease that results from mutations in the CF transmembrane conductance regulator (CFTR) gene. The effect of interventions aimed at correcting the CF electrophysiologic phenotype has been primarily measured using in vitro methods in gastrointestinal and respiratory epithelia. A reliable in vivo assay of CFTR function would be of great value in the investigation of pharmacologic interventions for CF mouse models. We performed the in vivo rectal potential difference (RPD) assay on three different mouse models. We then compared the in vivo data with the results obtained using the in vitro Ussing chamber method. The results from the in vitro method correlated closely with the results acquired using the in vivo method and were reproducible. The data suggest that the in vivo RPD assay is a reliable assay of functional CFTR expression in CF mouse models.

Authors+Show Affiliations

Department of Pediatrics, Yale University School of Medicine, New Haven, CT 06510, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Evaluation Study
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

18043508

Citation

Weiner, Scott A., et al. "Rectal Potential Difference and the Functional Expression of CFTR in the Gastrointestinal Epithelia in Cystic Fibrosis Mouse Models." Pediatric Research, vol. 63, no. 1, 2008, pp. 73-8.
Weiner SA, Caputo C, Bruscia E, et al. Rectal potential difference and the functional expression of CFTR in the gastrointestinal epithelia in cystic fibrosis mouse models. Pediatr Res. 2008;63(1):73-8.
Weiner, S. A., Caputo, C., Bruscia, E., Ferreira, E. C., Price, J. E., Krause, D. S., & Egan, M. E. (2008). Rectal potential difference and the functional expression of CFTR in the gastrointestinal epithelia in cystic fibrosis mouse models. Pediatric Research, 63(1), pp. 73-8.
Weiner SA, et al. Rectal Potential Difference and the Functional Expression of CFTR in the Gastrointestinal Epithelia in Cystic Fibrosis Mouse Models. Pediatr Res. 2008;63(1):73-8. PubMed PMID: 18043508.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Rectal potential difference and the functional expression of CFTR in the gastrointestinal epithelia in cystic fibrosis mouse models. AU - Weiner,Scott A, AU - Caputo,Christina, AU - Bruscia,Emanuela, AU - Ferreira,Elisa C, AU - Price,Joanna E, AU - Krause,Diane S, AU - Egan,Marie E, PY - 2007/11/29/pubmed PY - 2008/2/8/medline PY - 2007/11/29/entrez SP - 73 EP - 8 JF - Pediatric research JO - Pediatr. Res. VL - 63 IS - 1 N2 - Cystic fibrosis (CF) is an autosomal recessive disease that results from mutations in the CF transmembrane conductance regulator (CFTR) gene. The effect of interventions aimed at correcting the CF electrophysiologic phenotype has been primarily measured using in vitro methods in gastrointestinal and respiratory epithelia. A reliable in vivo assay of CFTR function would be of great value in the investigation of pharmacologic interventions for CF mouse models. We performed the in vivo rectal potential difference (RPD) assay on three different mouse models. We then compared the in vivo data with the results obtained using the in vitro Ussing chamber method. The results from the in vitro method correlated closely with the results acquired using the in vivo method and were reproducible. The data suggest that the in vivo RPD assay is a reliable assay of functional CFTR expression in CF mouse models. SN - 0031-3998 UR - https://www.unboundmedicine.com/medline/citation/18043508/Rectal_potential_difference_and_the_functional_expression_of_CFTR_in_the_gastrointestinal_epithelia_in_cystic_fibrosis_mouse_models_ L2 - http://dx.doi.org/10.1203/PDR.0b013e31815b4bc6 DB - PRIME DP - Unbound Medicine ER -