DIFFERENTIAL EXPRESSION OF ORCC AND CFTR INDUCED BY LOW-TEMPERATURE IN CF AIRWAY EPITHELIAL-CELLS

被引:41
作者
EGAN, ME
SCHWIEBERT, EM
GUGGINO, WB
机构
[1] JOHNS HOPKINS UNIV, SCH MED, DEPT PHYSIOL, BALTIMORE, MD 21205 USA
[2] JOHNS HOPKINS UNIV, SCH MED, DEPT PEDIAT, BALTIMORE, MD 21205 USA
来源
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY | 1995年 / 268卷 / 01期
关键词
CYSTIC FIBROSIS TRANSMEMBRANE REGULATOR PROTEIN; CHLORIDE CHANNELS; PATCH CLAMPING; DELTA-F508-CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR;
D O I
10.1152/ajpcell.1995.268.1.C243
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
When nonepithelial cell types expressing the Delta F508-cystic fibrosis transmembrane conductance regulator (CFTR) mutation are grown at reduced temperatures, the mutant protein can be properly processed. The effect of low temperatures on Cl- channel activity in airway epithelial cells that endogenously express the Delta F508-CFTR mutation has not been investigated. Therefore, we examined the effect of incubation temperature on both CFTR and outwardly rectifying Cl- channel (ORCC) activity in normal, in cystic fibrosis (CF)-affected, and in wild-type CFTR-complemented CF airway epithelia with use of a combination of inside-out and whole cell patch-clamp recording, Cl-36(-) efflux assays, and immunocytochemistry. We report that incubation of CF-affected airway epithelial cells at 25-27 degrees C is associated with the appearance of a protein kinase A-stimulated CFTR-like Cl- conductance. In addition to the appearance of CFTR Cl- channel activity, there is, however, a decrease in the number of active ORCC when cells are grown at 25-27 degrees C, suggesting that the decrease in incubation temperature may be associated with multiple alterations in ion channel expression and/or regulation in airway epithelial cells.
引用
收藏
页码:C243 / C251
页数:9
相关论文
共 34 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]  
Boat TF., 1989, CYSTIC FIBROSIS META, V6th, P2649
[4]   NA+ TRANSPORT IN CYSTIC-FIBROSIS RESPIRATORY EPITHELIA - ABNORMAL BASAL RATE AND RESPONSE TO ADENYLATE-CYCLASE ACTIVATION [J].
BOUCHER, RC ;
STUTTS, MJ ;
KNOWLES, MR ;
CANTLEY, L ;
GATZY, JT .
JOURNAL OF CLINICAL INVESTIGATION, 1986, 78 (05) :1245-1252
[5]   REGULATION OF PLASMA-MEMBRANE RECYCLING BY CFTR [J].
BRADBURY, NA ;
JILLING, T ;
BERTA, G ;
SORSCHER, EJ ;
BRIDGES, RJ ;
KIRK, KL .
SCIENCE, 1992, 256 (5056) :530-532
[6]  
CHENG SH, 1990, CELL, V63, P527
[7]   IMMUNOCYTOCHEMICAL LOCALIZATION OF THE CYSTIC-FIBROSIS GENE-PRODUCT CFTR [J].
CRAWFORD, I ;
MALONEY, PC ;
ZEITLIN, PL ;
GUGGINO, WB ;
HYDE, SC ;
TURLEY, H ;
GATTER, KC ;
HARRIS, A ;
HIGGINS, CF .
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1991, 88 (20) :9262-9266
[8]   ALTERED CHLORIDE-ION CHANNEL KINETICS ASSOCIATED WITH THE DELTA-F508 CYSTIC-FIBROSIS MUTATION [J].
DALEMANS, W ;
BARBRY, P ;
CHAMPIGNY, G ;
JALLAT, S ;
DOTT, K ;
DREYER, D ;
CRYSTAL, RG ;
PAVIRANI, A ;
LECOCQ, JP ;
LAZDUNSKI, M .
NATURE, 1991, 354 (6354) :526-528
[9]   PROCESSING OF MUTANT CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR IS TEMPERATURE-SENSITIVE [J].
DENNING, GM ;
ANDERSON, MP ;
AMARA, JF ;
MARSHALL, J ;
SMITH, AE ;
WELSH, MJ .
NATURE, 1992, 358 (6389) :761-764
[10]   ABNORMAL LOCALIZATION OF CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR IN PRIMARY CULTURES OF CYSTIC-FIBROSIS AIRWAY EPITHELIA [J].
DENNING, GM ;
OSTEDGAARD, LS ;
WELSH, MJ .
JOURNAL OF CELL BIOLOGY, 1992, 118 (03) :551-559