In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator (original) (raw)

Nature volume 353, pages 434–437 (1991)Cite this article

Abstract

CYSTIC fibrosis (CF) is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR)1–3. The principal manifestations of CF include increased concentration of Cl− in exocrine gland secretions4,5, pancreatic insufficiency, chronic lung disease, intestinal blockage and malabsorption of fat5,6, and male and female infertility7. Insight into the function of CFTR can be gained by correlating its cell-specific expression with the physiology of those cells and with CF pathology. Determination of CFTR messenger RNA in rat tissues by in situ hybridization shows that it is specifically expressed in the ductal cells of the pancreas and the salivary glands. In the intestine, decreasing gradients of expression of the CFTR gene are observed on both the crypt–villus and the proximal–distal axes. This expression is consistent with CFTR being responsible for bidirectional Cl− transport, secretion in the intestinal crypts8 and reabsorption in the silivary gland ducts4, and suggests that in these tissues CFTR functions as a regulated Cl− channel. In the lung, a broad band of hybridization includes the mucosa and submucosa of the bronchi and bronchioles. In the testis, CFTR expression is regulated during the cycle of the seminiferous epithelium. Post-meiotic expression is maximal in the round spermatids of stages VII and VIII, suggesting that CFTR plays a critical role in spermatogenesis and that deficiency of this function contributes to CF male infertility.

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Authors and Affiliations

  1. Department of Genetics, Research Institute, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, M5G 1X8, Canada
    Ann E. O. Trezíse & Manuel Buchwald
  2. Department of Molecular and Medical Genetics, University of Toronto, Toronto, Ontario, M5S 1A, Canada
    Manuel Buchwald

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  1. Ann E. O. Trezíse
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  2. Manuel Buchwald
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Trezíse, A., Buchwald, M. In vivo cell-specific expression of the cystic fibrosis transmembrane conductance regulator.Nature 353, 434–437 (1991). https://doi.org/10.1038/353434a0

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