ABCMdb

PMID: 18458238

Griesenbach U, Smith SN, Farley R, Singh C, Alton EW
Validation of nasal potential difference measurements in gut-corrected CF knockout mice.
Am J Respir Cell Mol Biol. 2008 Oct;39(4):490-6. Epub 2008 May 5., [PubMed]

Sentences

No. Mutations Sentence Comment

15 ABCC7 p.Gly551Asp In these mice the cystic fibrosis transmembrane conductance regulator (Cftr) gene, which encodes an epithelial chloride channel, is either completely knocked out (nulls) or naturally occurring mutations such as DF508 or G551D have been introduced (4), leading to altered chloride and sodium transport across epithelial cells. Login to comment 21 ABCC7 p.Gly551Asp To assess the effects of gene and small molecule therapy on the respiratory tract, it is useful to use a strain that has very low, or absent, residual CFTR function such as the UNC-null (7), G551D (8), or DF508 mice (9). Login to comment 42 ABCC7 p.Gly551Asp ABCC7 p.Gly551Asp E-mail: u.griesenbach@imperial.ac.uk Am J Respir Cell Mol Biol Vol 39. pp 490-496, 2008 Originally Published in Press as DOI: 10.1165/rcmb.2007-0385OC on May 5, 2008 Internet address: www.atsjournals.org strain dependent, we therefore, first compared PD in C57Bl/6 congenic G551D CF mice and C57Bl/6 wild-type mice, and subsequently compared PD in C57Bl/6 congenic G551D and FABp-CF mice. Login to comment 50 ABCC7 p.Gly551Asp MATERIALS AND METHODS Mouse Models Male and female wild-type C57Bl/6 mice and G551D CF knockout mice (8) were purchased from Charles River UK (Margate, Kent, UK). Login to comment 98 ABCC7 p.Gly551Asp RESULTS Comparison of Nasal PD in Wild-Type, GD-B6, and FABp-CF-Knockout Mice Transepithelial nasal potential difference was measured in C57Bl/6 wild-type (WT-B6), C57Bl/6 congenic G551D (GD-B6), and FABp-CF mice. Login to comment 101 ABCC7 p.Gly551Asp There was no difference in nasal PD between G551D and FABp CF mice (Figure 1). Login to comment 112 ABCC7 p.Gly551Asp Nasal PD was measured in wild-type C57Bl/6 mice (WT-B6), C56Bl/6 congenic G551D CF knockout mice (GD-B6), and gut-corrected CF knockout mice (FABp). Login to comment 178 ABCC7 p.Gly551Asp We were aware that the absence of genetically matched wild-type littermates might affect the interpretation of our results. To address this point, we compared nasal PDs of FABp-CF mice, C57Bl/6 congenic G551D mice, and C57Bl/6 WT mice. Login to comment 179 ABCC7 p.Gly551Asp Importantly, both strains of CF mice were significantly different from WT for all PD-related measurements, and there was no difference between FABp and G551D CF mice. Login to comment 181 ABCC7 p.Gly551Asp Animal numbers in the G551D cohort were lower than in WT and FABp cohorts due to the restricted availability and poor health of these mice. Login to comment 200 ABCC7 p.Arg117His Most importantly, patients with ''mild`` CF mutations such as R117H who have some residual CFTR function (19) generally have less severe lung disease. Login to comment