Widdicombe J H, Wine J J
Cystic Fibrosis Research Center, University of California, San Francisco 94143.
Trends Biochem Sci. 1991 Dec;16(12):474-7. doi: 10.1016/0968-0004(91)90183-v.
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.
最近的证据有力地表明，囊性纤维化基因产物（CFTR）是一种氯离子通道。然而，其特性与先前被认为在囊性纤维化中存在缺陷的30 - 50 pS外向整流通道不同。囊性纤维化缺陷的多效性影响，如气道钠吸收增加和黏液硫酸化，是继发于氯离子传导降低，还是反映了CFTR的其他功能，目前仍不确定。
