OUTWARDLY RECTIFYING CHLORIDE CHANNELS AND CF - A DIVORCE AND REMARRIAGE

被引:41
作者
GUGGINO, WB
机构
[1] Johns Hopkins University School of Medicine, Baltimore, 21205
关键词
CHLORIDE CHANNELS; CF; OUTWARDLY RECTIFYING CHLORIDE CHANNELS; CAMP; CFTR; REVIEW;
D O I
10.1007/BF00768065
中图分类号
Q6 [生物物理学];
学科分类号
071011 ;
摘要
Outwardly rectifying Cl- channels were originally thought to be the central element in cystic fibrosis. The role of these channels in CF was questioned to such an extent that doubts were raised about the validity of the original experiments. Recent data reestablishes a role for outwardly rectifying Cl- channels (ORCC) in CF and suggests that the protein encoded by the CF gene, the cystic fibrosis transmembrane regulator (CFTR), can effect the regulation of more than one channel in the airway. This minireview deals with the rise, fall, and resurrection of the role of outwardly rectifying Cl- channels in CF.
引用
收藏
页码:27 / 35
页数:9
相关论文
共 54 条
  • [1] FATTY-ACIDS INHIBIT APICAL MEMBRANE CHLORIDE CHANNELS IN AIRWAY EPITHELIA
    ANDERSON, MP
    WELSH, MJ
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1990, 87 (18) : 7334 - 7338
  • [2] DEMONSTRATION THAT CFTR IS A CHLORIDE CHANNEL BY ALTERATION OF ITS ANION SELECTIVITY
    ANDERSON, MP
    GREGORY, RJ
    THOMPSON, S
    SOUZA, DW
    PAUL, S
    MULLIGAN, RC
    SMITH, AE
    WELSH, MJ
    [J]. SCIENCE, 1991, 253 (5016) : 202 - 205
  • [3] GENERATION OF CAMP-ACTIVATED CHLORIDE CURRENTS BY EXPRESSION OF CFTR
    ANDERSON, MP
    RICH, DP
    GREGORY, RJ
    SMITH, AE
    WELSH, MJ
    [J]. SCIENCE, 1991, 251 (4994) : 679 - 682
  • [4] DEFECTIVE ACIDIFICATION OF INTRACELLULAR ORGANELLES IN CYSTIC-FIBROSIS
    BARASCH, J
    KISS, B
    PRINCE, A
    SAIMAN, L
    GRUENERT, D
    ALAWQATI, Q
    [J]. NATURE, 1991, 352 (6330) : 70 - 73
  • [5] PURIFICATION AND FUNCTIONAL RECONSTITUTION OF THE CYSTIC-FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (CFTR)
    BEAR, CE
    LI, CH
    KARTNER, N
    BRIDGES, RJ
    JENSEN, TJ
    RAMJEESINGH, M
    RIORDAN, JR
    [J]. CELL, 1992, 68 (04) : 809 - 818
  • [6] BEAR CE, 1991, J BIOL CHEM, V266, P19142
  • [7] A CAMP-REGULATED CHLORIDE CHANNEL IN LYMPHOCYTES THAT IS AFFECTED IN CYSTIC-FIBROSIS
    CHEN, JH
    SCHULMAN, H
    GARDNER, P
    [J]. SCIENCE, 1989, 243 (4891) : 657 - 660
  • [8] DEFECTIVE EPITHELIAL CHLORIDE TRANSPORT IN A GENE-TARGETED MOUSE MODEL OF CYSTIC-FIBROSIS
    CLARKE, LL
    GRUBB, BR
    GABRIEL, SE
    SMITHIES, O
    KOLLER, BH
    BOUCHER, RC
    [J]. SCIENCE, 1992, 257 (5073) : 1125 - 1128
  • [9] SEPARATE CL- CONDUCTANCES ACTIVATED BY CAMP AND CA-2+ IN CL--SECRETING EPITHELIAL-CELLS
    CLIFF, WH
    FRIZZELL, RA
    [J]. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1990, 87 (13) : 4956 - 4960
  • [10] CYSTIC-FIBROSIS - MOLECULAR-BIOLOGY AND THERAPEUTIC IMPLICATIONS
    COLLINS, FS
    [J]. SCIENCE, 1992, 256 (5058) : 774 - 779