Abstract
It is now well established that cystic fibrosis is expressed physiologically as defects in certain fluid and electrolyte transport systems that are highly active in exocrine tissues (Quinton, 1983; Knowles et al., 1983; Kopelman et al., 1982; Orlando et al., 1989). More specifically, two basic physiological defects have been demonstrated directly in at least two of the organ systems characteristically affected in cystic fibrosis -the sweat glands and the airways. The two defects in these affected epithelia appear as 1) an inherently reduced permeability to Cl-(Quinton, 1983; Widdicombe et al., 1985; Knowles, 1983), and 2) a failure of fluid secretion to respond to β-adrenergic stimulation (Sato and Sato, 1984; Widdicombe et al., 1985). The refractory response to β-adrenergic stimulation is most clearly seen in secretory functions and is thought to be due to an inactivatable chloride channel (Welsh, 1986; Frizzell, 1986). While other defects have been associated with cystic fibrosis, e.g., increased Na+ permeability (Knowles et al., 1983; Boucher et al., 1986; Willumsen and Boucher, 1989), abnormal sulfate metabolism (Cheng et al., 1985), and abnormal calcium levels (Sorcher and Beslow, 1982; Shapiro et al., 1980), the basic physiological abnormality in the disease seems to center upon chloride impermeability.
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Quinton, P.M., Reddy, M.M. (1991). Regulation of Absorption in the Human Sweat Duct. In: Tsui, LC., Romeo, G., Greger, R., Gorini, S. (eds) The Identification of the CF (Cystic Fibrosis) Gene. Advances in Experimental Medicine and Biology, vol 290. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-5934-0_17
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DOI: https://doi.org/10.1007/978-1-4684-5934-0_17
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