Review
doi: 10.1016/0968-0004(91)90183-v.
The basic defect in cystic fibrosis
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- PMID: 1723552
- DOI: 10.1016/0968-0004(91)90183-v
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Review
The basic defect in cystic fibrosis
Trends Biochem Sci.
1991 Dec.
Abstract
Recent evidence strongly suggests that the cystic fibrosis gene product (CFTR) is a Cl- channel. Its properties, however, differ from those of a 30-50 pS outwardly rectifying channel previously implicated as defective in cystic fibrosis. It is still uncertain whether the pleiotropic effects of the CF defect, such as increased airway Na+ absorption and mucus sulfation, are secondary to reduced Cl- conductance, or reflect additional functions of CFTR.
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