Rectal potential difference and the functional expression of CFTR in the gastrointestinal epithelia in cystic fibrosis mouse models.
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.
Department of Pediatrics, Yale University School of Medicine, New Haven, CT 06510, USA.
Scott A
SA
Weiner
Christina
C
Caputo
Emanuela
E
Bruscia
Elisa C
EC
Ferreira
Joanna E
JE
Price
Diane S
DS
Krause
Marie E
ME
Egan
D000818:Animals; D001565:Benzoates / Q000494:pharmacology; D005576:Colforsin / Q000494:pharmacology; D003106:Colon / Q000187:drug effects / Q000378:metabolism / Q000503:physiopathology*; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000187:drug effects / Q000235:genetics / Q000378:metabolism*; D004195:Disease Models, Animal; D005838:Genotype; D007413:Intestinal Mucosa / Q000187:drug effects / Q000378:metabolism / Q000503:physiopathology*; D008564:Membrane Potentials; D051379:Mice; D018181:Mice, Inbred CFTR; D008817:Mice, Mutant Strains; D010641:Phenotype; D012007:Rectum / Q000187:drug effects / Q000378:metabolism / Q000503:physiopathology*; D015203:Reproducibility of Results; D053778:Thiazolidines / Q000494:pharmacology
C482900:3-((3-trifluoromethyl)phenyl)-5-((3-carboxyphenyl)methylene)-2-thioxo-4-thiazolidinone; D001565:Benzoates; D053778:Thiazolidines; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D005576:Colforsin