Development, Vol 113, Issue 1 305-310, Copyright © 1991 by Company of Biologists

JOURNAL ARTICLES

Expression of the cystic fibrosis gene in human development

A Harris, G Chalkley, S Goodman and L Coleman
Paediatric Research Unit, United Medical School, Guy's Hospital, London, UK.

The specialised epithelia lining the respiratory tract, pancreatic ducts, male genital ducts and sweat gland ducts are defective in the severe inherited disease, cystic fibrosis (CF). We have looked at the expression of the CF gene in human fetal tissues to throw light on the development of function in specialised ductal epithelia and to determine the age of onset of the CF disease process. The CF gene is already seen to be transcribed in mid-trimester fetal lung, pancreas and male genital ducts. Hence, by this developmental stage, and before they are fully differentiated, these epithelia have the capability to perform important transport functions. Epithelial cell cultures derived from fetal pancreas and male genital ducts maintain expression of the CF gene in vitro and so form good models for analysing CF gene function and differentiation of these specialised epithelia.

This article has been cited by other articles:

				T. T. Turner De Graaf's Thread: The Human Epididymis J Androl, May 1, 2008; 29(3): 237 - 250. [Abstract] [Full Text] [PDF]

				Y. Xu, C. Liu, J. C. Clark, and J. A. Whitsett Functional Genomic Responses to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and CFTR{Delta}508 in the Lung J. Biol. Chem., April 21, 2006; 281(16): 11279 - 11291. [Abstract] [Full Text] [PDF]

				A. Ben-Chetrit, M. Antenos, A. Jurisicova, E. A. Pasyk, D. Chitayat, J.K. Foskett, and R. F. Casper Expression of cystic fibrosis transmembrane conductance regulator during early human embryo development Mol. Hum. Reprod., August 1, 2002; 8(8): 758 - 764. [Abstract] [Full Text] [PDF]

				F. C. Broackes-Carter, N. Mouchel, D. Gill, S. Hyde, J. Bassett, and A. Harris Temporal regulation of CFTR expression during ovine lung development: implications for CF gene therapy Hum. Mol. Genet., January 1, 2002; 11(2): 125 - 131. [Abstract] [Full Text] [PDF]

				M. Horster Embryonic epithelial membrane transporters Am J Physiol Renal Physiol, December 1, 2000; 279(6): F982 - F996. [Abstract] [Full Text] [PDF]

				J. E. Larson, J. B. Delcarpio, M. M. Farberman, S. L. Morrow, and J. C. Cohen CFTR modulates lung secretory cell proliferation and differentiation Am J Physiol Lung Cell Mol Physiol, August 1, 2000; 279(2): L333 - L341. [Abstract] [Full Text] [PDF]

				M. Bertog, D. J. Smith, A. Bielfeld-Ackermann, J. Bassett, D. J. P. Ferguson, C. Korbmacher, and A. Harris Ovine male genital duct epithelial cells differentiate in vitro and express functional CFTR and ENaC Am J Physiol Cell Physiol, May 1, 2000; 278(5): C885 - C894. [Abstract] [Full Text] [PDF]

				M.-C. Romey, N. Pallares-Ruiz, A. Mange, C. Mettling, R. Peytavi, J. Demaille, and M. Claustres A Naturally Occurring Sequence Variation That Creates a YY1 Element Is Associated with Increased Cystic Fibrosis Transmembrane Conductance Regulator Gene Expression J. Biol. Chem., February 4, 2000; 275(5): 3561 - 3567. [Abstract] [Full Text] [PDF]

				A. HARRIS The Duct Cell in Cystic Fibrosis Ann. N.Y. Acad. Sci., June 30, 1999; 880(1): 17 - 30. [Abstract] [Full Text] [PDF]

				O. CHIBA-FALEK, R. B. PARAD, E. KEREM, and B. KEREM Variable Levels of Normal RNA in Different Fetal Organs Carrying a Cystic Fibrosis Transmembrane Conductance Regulator Splicing Mutation Am. J. Respir. Crit. Care Med., June 1, 1999; 159(6): 1998 - 2002. [Abstract] [Full Text]

				J. M. PILEWSKI and R. A. FRIZZELL Role of CFTR in Airway Disease Physiol Rev, January 1, 1999; 79(1): 215 - 255. [Abstract] [Full Text] [PDF]

				A. N. Smith, M. L. Barth, T. L. McDowell, D. S. Moulin, H. N. Nuthall, M. A. Hollingsworth, and A. Harris A Regulatory Element in Intron 1 of the Cystic Fibrosis Transmembrane Conductance Regulator Gene J. Biol. Chem., April 26, 1996; 271(17): 9947 - 9954. [Abstract] [Full Text] [PDF]

				E. B. Mallow, A. Harris, N. Salzman, J. P. Russell, R. J. DeBerardinis, E. Ruchelli, and C. L. Bevins Human Enteric Defensins J. Biol. Chem., February 23, 1996; 271(8): 4038 - 4045. [Abstract] [Full Text] [PDF]

				J. Chambers, M. Hollingsworth, A. Trezise, and A Harris Developmental expression of mucin genes MUC1 and MUC2 J. Cell Sci., January 2, 1994; 107(2): 413 - 424. [Abstract] [PDF]

				J. Chambers and A Harris Expression of the cystic fibrosis gene and the major pancreatic mucin gene, MUC1, in human ductal epithelial cells J. Cell Sci., January 6, 1993; 105(2): 417 - 422. [Abstract] [PDF]