THE BASIC DEFECT IN CYSTIC-FIBROSIS

被引：32

作者：

WIDDICOMBE, JH ^{[1
]}

WINE, JJ ^{[1
]}

机构：

[1] STANFORD UNIV,STANFORD,CA 94305

来源：

TRENDS IN BIOCHEMICAL SCIENCES | 1991年 / 16卷 / 12期

关键词：

D O I：

10.1016/0968-0004(91)90183-V

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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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收藏

页码：474 / 477

页数：4

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