ABCMdb

PMID: 12820661

Oceandy D, McMorran B, Schreiber R, Wainwright BJ, Kunzelmann K
GFP-tagged CFTR transgene is functional in the G551D cystic fibrosis mouse colon.
J Membr Biol. 2003 Apr 1;192(3):159-67., 2003-04-01 [PubMed]

Sentences

No. Mutations Sentence Comment

1 ABCC7 p.Gly551Asp 2003 GFP-Tagged CFTR Transgene is Functional in the G551D Cystic Fibrosis Mouse Colon D. Oceandy l, B. McMorran l, R. Schreiber2, B.J. Wainwright1, K. Kunzelmann2 IInstitute for Molecular Biosciences, The University of Queensland, St. Lucia QLD 4072, Australia 2Institut ffir Physiologic, Universit~it Regensburg, UniversitfitstraBe 31, D-93053 Regensburg Received: 25 September 2002/Revised: 13 December 2003 Abstract. Login to comment 52 ABCC7 p.Gly551Asp The transgenic founder was bred with CD1 mice and the transgenic offspring were crossed with G551D CF mice [5] to generate bitransgenic animals carrying the K18-GFP-CFTR transgene and the cftr c'SsjD allele. Login to comment 54 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp The four strains used in this study, ~;[?r+/+ (wt), ~JtrG551D/G551D (CF), QQrG5slD/G551D K18-CFTR ~ (K18) [30], and ~ftr ~ K18-GFP-CFTR +/ (GFP) have equivalent genetic backgrounds (a mixture of CDI, C57-B6 and 129). Login to comment 80 ABCC7 p.Gly551Asp Results PHENOTYPIC ASPECT OF cftrG551D/G551D K18-GFP-CFTR +/- MICE Typically cftrGSslD/Gs51D have a significant perinatal mortality primarily due to meconium ileus and sur- Fig. 1. Login to comment 85 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp Bitransgenic cftr G551D/G551D (CF) mice carrying the K18-GFP-CFTR transgene showed in general a good viability, which exceeded that of the cftr G551D/G551D mice. Login to comment 87 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp The bitransgenic CF mice were also significantly heavier than the cftr G551D/G551D mice and similar to non-transgenic cftr +/+ mice. Login to comment 88 ABCC7 p.Gly551Asp Mean weights (+ standard deviation) of males at 10 weeks of age were 33.0-4-2.5 g, 24.8 + 3.0 g and 34.7 • 2.9 g for bitransgenic cftr c551D/C55~I~j, cftr G551D/G551I~ and cftr +/+ mice respectively (n > 5), indicating that the transgene was complementing the gastrointestinal defect at the broad phenotypic level. Login to comment 89 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp EXPRESSIONOF THE GFP-CFTR TRANSGENEIN THE COLON Expression of the human CFTR transgene could be readily detected in the colon of bitransgenic cftr G551D/G551D K18-GFP-CFTR +! Login to comment 107 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp TRANSPORT PROPERTIES OF THE COLONIC EPITHELIUM AND IN THE TRACHEA OF cftr G551D/G551D K18-GFP-CFTR +/- MICE We examined the ion transport in the colonic epithelium of GFP mice and compared the results with those obtained in the colon of cftr cSslD/G551D mice, wt mice and bitransgenic cftr ~SslD/~Ssm K18- Fig. 4. Login to comment 132 ABCC7 p.Gly551Asp After stimulation with IBMX and forskolin and activation of CFTR, a negative voltage deflection is induced in wt, GFP and K18 mice but not in G551D animals (Figs. 5 and 6). Login to comment 152 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp We were interested in studying expression of a GFP-CFTR G551D/G551D transgene in cftr mice. Login to comment 154 ABCC7 p.Gly551Asp The present results indicate expression of transcripts and protein of GFP-CFTR in G55 ID/G551D bitransgenic cftr K18-GFP-CFTR mice. Login to comment 180 ABCC7 p.Gly551Asp Although multiple and also reciprocal interactions between CFTR and other ion channels such as ENaC have been described in several previous studies [reviewed in 20, 35], the reason and functional mechanism for the decreased Na + absorption in the colon of G551D animals, and the increase in Na+ transport after expression of K18-GFP-CFTR remain obscure. Login to comment 181 ABCC7 p.Gly551Asp However, it seems important to note that the colon of G551D (CF) mice is smaller than that of the other animals, that it demonstrates an altered quality and is difficult to prepare for Ussing chamber recordings. Login to comment