Is cystic fibrosis lung disease caused by abnormal ion composition or abnormal volume?

被引:9
作者
Krouse, ME [1 ]
机构
[1] Stanford Univ, Cyst Fibrosis Res Lab, Stanford, CA 94305 USA
关键词
D O I
10.1085/jgp.118.2.219
中图分类号
Q4 [生理学];
学科分类号
071003 ;
摘要
引用
收藏
页码:219 / 221
页数:3
相关论文
共 25 条
[1]   NUCLEOSIDE TRIPHOSPHATES ARE REQUIRED TO OPEN THE CFTR CHLORIDE CHANNEL [J].
ANDERSON, MP ;
BERGER, HA ;
RICH, DP ;
GREGORY, RJ ;
SMITH, AE ;
WELSH, MJ .
CELL, 1991, 67 (04) :775-784
[2]   PURIFICATION AND FUNCTIONAL RECONSTITUTION OF THE CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (CFTR) [J].
BEAR, CE ;
LI, CH ;
KARTNER, N ;
BRIDGES, RJ ;
JENSEN, TJ ;
RAMJEESINGH, M ;
RIORDAN, JR .
CELL, 1992, 68 (04) :809-818
[3]   Molecular insights into the physiology of the 'thin film' of airway surface liquid [J].
Boucher, RC .
JOURNAL OF PHYSIOLOGY-LONDON, 1999, 516 (03) :631-638
[4]   PHOSPHORYLATION OF THE R-DOMAIN BY CAMP-DEPENDENT PROTEIN-KINASE REGULATES THE CFTR CHLORIDE CHANNEL [J].
CHENG, SH ;
RICH, DP ;
MARSHALL, J ;
GREGORY, RJ ;
WELSH, MJ ;
SMITH, AE .
CELL, 1991, 66 (05) :1027-1036
[5]   Cystic fibrosis [J].
Davis, PB ;
Drumm, M ;
Konstan, MW .
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 1996, 154 (05) :1229-1256
[6]   REGULATION OF THE GATING OF CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR CL CHANNELS BY PHOSPHORYLATION AND ATP HYDROLYSIS [J].
HWANG, TC ;
NAGEL, G ;
NAIRN, AC ;
GADSBY, DC .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1994, 91 (11) :4698-4702
[7]   ALTERED FLUID TRANSPORT ACROSS AIRWAY EPITHELIUM IN CYSTIC-FIBROSIS [J].
JIANG, CW ;
FINKBEINER, WE ;
WIDDICOMBE, JH ;
MCCRAY, PB ;
MILLER, SS .
SCIENCE, 1993, 262 (5132) :424-427
[8]   ELEMENTAL COMPOSITION OF HUMAN AIRWAY SURFACE FLUID IN HEALTHY AND DISEASED AIRWAYS [J].
JORIS, L ;
DAB, I ;
QUINTON, PM .
AMERICAN REVIEW OF RESPIRATORY DISEASE, 1993, 148 (06) :1633-1637
[9]   Ion composition of airway surface liquid of patients with cystic fibrosis as compared with normal and disease-control subjects [J].
Knowles, MR ;
Robinson, JM ;
Wood, RE ;
Pue, CA ;
Mentz, WM ;
Wager, GC ;
Gatzy, JT ;
Boucher, RC .
JOURNAL OF CLINICAL INVESTIGATION, 1997, 100 (10) :2588-2595
[10]  
KNOWLES MR, 1986, CLIN CHEST MED, V7, P285