CFTR, A CHANNEL WITH THE STRUCTURE OF A TRANSPORTER

被引:37
作者
RIORDAN, JR
CHANG, XB
机构
[1] UNIV TORONTO,DEPT BIOCHEM,TORONTO M5S 1A1,ONTARIO,CANADA
[2] UNIV TORONTO,DEPT CLIN BIOCHEM,TORONTO M5S 1A1,ONTARIO,CANADA
关键词
CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; MEMBRANE TRANSPORT;
D O I
10.1016/0005-2728(92)90230-Y
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
The cystic fibrosis transmembrane conductance regulator (CFTR) belongs to a superfamily of active transport molecules. However, when expressed in a wide variety of heterologous cell systems and when purified to homogeneity and reconstituted in planar lipid bilayers, it exhibits low conductance chloride channel activity. We postulate that the active transport capability of the molecule has been adapted to provide very stringent metabolic control of this channel which is responsible for chloride secretion and hydration of wet epithelial surfaces.
引用
收藏
页码:221 / 222
页数:2
相关论文
共 18 条
[1]   GENERATION OF CAMP-ACTIVATED CHLORIDE CURRENTS BY EXPRESSION OF CFTR [J].
ANDERSON, MP ;
RICH, DP ;
GREGORY, RJ ;
SMITH, AE ;
WELSH, MJ .
SCIENCE, 1991, 251 (4994) :679-682
[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]  
BEAR CE, 1991, J BIOL CHEM, V266, P19142
[4]   STRUCTURE AND EXPRESSION OF THE HUMAN MDR (P-GLYCOPROTEIN) GENE FAMILY [J].
CHIN, JE ;
SOFFIR, R ;
NOONAN, KE ;
CHOI, K ;
RONINSON, IB .
MOLECULAR AND CELLULAR BIOLOGY, 1989, 9 (09) :3808-3820
[5]   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
[6]   CORRECTION OF THE CYSTIC-FIBROSIS DEFECT INVITRO BY RETROVIRUS-MEDIATED GENE-TRANSFER [J].
DRUMM, ML ;
POPE, HA ;
CLIFF, WH ;
ROMMENS, JM ;
MARVIN, SA ;
TSUI, LC ;
COLLINS, FS ;
FRIZZELL, RA ;
WILSON, JM .
CELL, 1990, 62 (06) :1227-1233
[7]   CHLORIDE CONDUCTANCE EXPRESSED BY DELTA-F508 AND OTHER MUTANT CFTRS IN XENOPUS OOCYTES [J].
DRUMM, ML ;
WILKINSON, DJ ;
SMIT, LS ;
WORRELL, RT ;
STRONG, TV ;
FRIZZELL, RA ;
DAWSON, DC ;
COLLINS, FS .
SCIENCE, 1991, 254 (5039) :1797-1799
[8]   THE BIOCHEMISTRY OF P-GLYCOPROTEIN-MEDIATED MULTIDRUG RESISTANCE [J].
ENDICOTT, JA ;
LING, V .
ANNUAL REVIEW OF BIOCHEMISTRY, 1989, 58 :137-171
[9]   EXPRESSION OF THE CYSTIC-FIBROSIS GENE IN NONEPITHELIAL INVERTEBRATE CELLS PRODUCES A REGULATED ANION CONDUCTANCE [J].
KARTNER, N ;
HANRAHAN, JW ;
JENSEN, TJ ;
NAISMITH, AL ;
SUN, SZ ;
ACKERLEY, CA ;
REYES, EF ;
TSUI, LC ;
ROMMENS, JM ;
BEAR, CE ;
RIORDAN, JR .
CELL, 1991, 64 (04) :681-691
[10]   IDENTIFICATION OF THE CYSTIC-FIBROSIS GENE - GENETIC-ANALYSIS [J].
KEREM, BS ;
ROMMENS, JM ;
BUCHANAN, JA ;
MARKIEWICZ, D ;
COX, TK ;
CHAKRAVARTI, A ;
BUCHWALD, M ;
TSUI, LC .
SCIENCE, 1989, 245 (4922) :1073-1080