Epithelial cell chloride channel activity correlates with improved airway function in cystic fibrosis patients with the major mutant: Delta F508

被引:0
作者
Kidd, JF
Bear, CE
机构
[1] Hosp Sick Children, Res Inst, Programme Struct Biol & Biochem, Toronto, ON M5G 1X8, Canada
[2] Fac Med, Dept Biochem, Toronto, ON M5G 1X8, Canada
[3] Fac Med, Dept Physiol, Toronto, ON M5G 1X8, Canada
关键词
D O I
10.1203/01.PDR.0000032982.37512.67
中图分类号
R72 [儿科学];
学科分类号
100202 ;
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页码:625 / 627
页数:3
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共 34 条
  • [11] ΔF508 CFTR protein expression in tissues from patients with cystic fibrosis
    Kälin, N
    Claass, A
    Sommer, M
    Puchelle, E
    Tümmler, B
    [J]. JOURNAL OF CLINICAL INVESTIGATION, 1999, 103 (10) : 1379 - 1389
  • [12] MISLOCALIZATION OF DELTA-F508 CFTR IN CYSTIC-FIBROSIS SWEAT GLAND
    KARTNER, N
    AUGUSTINAS, O
    JENSEN, TJ
    NAISMITH, AL
    RIORDAN, JR
    [J]. NATURE GENETICS, 1992, 1 (05) : 321 - 327
  • [13] Inflammation and cystic fibrosis pulmonary disease
    Kennedy, MJ
    [J]. PHARMACOTHERAPY, 2001, 21 (05): : 593 - 603
  • [14] KEREM B, 1989, SCIENCE, V245, P1437
  • [15] THE RELATION BETWEEN GENOTYPE AND PHENOTYPE IN CYSTIC-FIBROSIS - ANALYSIS OF THE MOST COMMON MUTATION (DELTA-F508)
    KEREM, E
    COREY, M
    KEREM, BS
    ROMMENS, J
    MARKIEWICZ, D
    LEVISON, H
    TSUI, LC
    DURIE, P
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1990, 323 (22) : 1517 - 1522
  • [16] KHAN TZ, 1995, AM J RESP CRIT CARE, V151, P1075
  • [17] THE CYSTIC-FIBROSIS MUTATION (DELTA-F508) DOES NOT INFLUENCE THE CHLORIDE CHANNEL ACTIVITY OF CFTR
    LI, CH
    RAMJEESINGH, M
    REYES, E
    JENSEN, T
    CHANG, XB
    ROMMENS, JM
    BEAR, CE
    [J]. NATURE GENETICS, 1993, 3 (04) : 311 - 316
  • [18] Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
    Matsui, H
    Grubb, BR
    Tarran, R
    Randell, SH
    Gatzy, JT
    Davis, CW
    Boucher, RC
    [J]. CELL, 1998, 95 (07) : 1005 - 1015
  • [19] Role of mutant CFTR in hypersusceptibility of cystic fibrosis patients to lung infections
    Pier, GB
    Grout, M
    Zaidi, TS
    Olsen, JC
    Johnson, LG
    Yankaskas, JR
    Goldberg, JB
    [J]. SCIENCE, 1996, 271 (5245) : 64 - 67
  • [20] Pilewski Joseph M., 1999, Physiological Reviews, V79, pS215