Skip Navigation

This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Search for citing articles in:
ISI Web of Science (23)
Right arrowRequest Permissions
Google Scholar
Right arrow Articles by Broackes-Carter, F. C.
Right arrow Articles by Harris, A.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Broackes-Carter, F. C.
Right arrow Articles by Harris, A.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Human Molecular Genetics, 2002, Vol. 11, No. 2 125-131
© 2002 Oxford University Press

Temporal regulation of CFTR expression during ovine lung development: implications for CF gene therapy

Fiona C. Broackes-Carter, Nathalie Mouchel, Deborah Gill1, Stephen Hyde1, John Bassett2 and Ann Harris+

Paediatric Molecular Genetics, Institute of Molecular Medicine, Oxford University, John Radcliffe Hospital, Oxford OX3 9DS, UK, 1Nuffield Department of Clinical Laboratory Sciences, Oxford University, John Radcliffe Hospital, Oxford OX3 9DU, UK and 2Growth and Development Unit, Field Laboratory, Oxford University, Oxford OX2 8QJ, UK

The cystic fibrosis transmembrane conductance regulator (CFTR) protein is a small conductance chloride ion channel that may interact directly with other channels including the epithelial sodium channel (ENaC). CFTR is known to be more abundant in the airway epithelium during the second trimester of human development than after birth. This could be a consequence of the change in function of the respiratory epithelium from chloride secretion to sodium absorption near term. Alternatively it might reflect an additional role for CFTR in the developing airway epithelium. Though the lung epithelia of CF fetuses and infants rarely show gross histological abnormalities, there is often evidence of inflammation. Our aim was to establish whether CFTR expression levels correlated with specific developmental stages or differentiated functions in the ovine fetal lung. We evaluated CFTR expression using a quantitative assay of mRNA at 14 time points through gestation and showed highest levels at the start of the second trimester followed by a gradual decline through to term. In contrast, ENaC expression increased from the start of the third trimester. These results support a role for CFTR in differentiation of the respiratory epithelium and suggest that its expression levels are not merely reflecting major changes in the sodium/chloride bulk flow close to term. These observations may have significant implications for the likely success of CF gene therapy in the postnatal lung.

+ To whom correspondence should be addressed. Fax: +44 1865 222626; Email: aharris@molbiol.ox.ac.uk


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
M. A. Carey, J. W. Card, J. W. Voltz, D. R. Germolec, K. S. Korach, and D. C. Zeldin
The impact of sex and sex hormones on lung physiology and disease: lessons from animal studies
Am J Physiol Lung Cell Mol Physiol, August 1, 2007; 293(2): L272 - L278.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Respir. Cell Mol. Bio.Home page
H. Davidson, G. McLachlan, A. Wilson, A. C. Boyd, A. Doherty, G. MacGregor, L. Davies, H. A. Painter, R. Coles, S. C. Hyde, et al.
Human-Specific Cystic Fibrosis Transmembrane Conductance Regulator Antibodies Detect In Vivo Gene Transfer to Ovine Airways
Am. J. Respir. Cell Mol. Biol., July 1, 2006; 35(1): 72 - 83.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Lung Cell. Mol. Physiol.Home page
J. R. Bourbon and A. Benachi
CFTR gene therapy, a method to rescue lung hypoplasia in congenital diaphragmatic hernia?
Am J Physiol Lung Cell Mol Physiol, July 1, 2006; 291(1): L1 - L3.
[Full Text] [PDF]

Home page
 Am. J. Respir. Cell Mol. Bio.Home page
M. O. Henke, A. Renner, R. M. Huber, M. C. Seeds, and B. K. Rubin
MUC5AC and MUC5B Mucins Are Decreased in Cystic Fibrosis Airway Secretions
Am. J. Respir. Cell Mol. Biol., July 1, 2004; 31(1): 86 - 91.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Respir. Cell Mol. Bio.Home page
K. W. Holmes, R. Hales, S. Chu, M. J. Maxwell, P. J. Mogayzel Jr., and P. L. Zeitlin
Modulation of Sp1 and Sp3 in Lung Epithelial Cells Regulates ClC-2 Chloride Channel Expression
Am. J. Respir. Cell Mol. Biol., October 1, 2003; 29(4): 499 - 505.
[Abstract] [Full Text] [PDF]

Home page
 Am. J. Physiol. Gastrointest. Liver Physiol.Home page
A. M. Hinojosa-Kurtzberg, M. E. V. Johansson, C. S. Madsen, G. C. Hansson, and S. J. Gendler
Novel MUC1 splice variants contribute to mucin overexpression in CFTR-deficient mice
Am J Physiol Gastrointest Liver Physiol, May 1, 2003; 284(5): G853 - G862.
[Abstract] [Full Text] [PDF]

Home page
 Hum Mol GenetHome page
N. Mouchel, F. Broackes-Carter, and A. Harris
Alternative 5' exons of the CFTR gene show developmental regulation
Hum. Mol. Genet., April 1, 2003; 12(7): 759 - 769.
[Abstract] [Full Text] [PDF]


Disclaimer:
Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.