Finally, mice with CF lung disease

被引:19
作者
Frizzell, RA [1 ]
Pilewski, JM
机构
[1] Univ Pittsburgh, Sch Med, Dept Cell Biol & Physiol, Pittsburgh, PA 15261 USA
[2] Univ Pittsburgh, Sch Med, Dept Med, Pittsburgh, PA 15261 USA
关键词
D O I
10.1038/nm0504-452
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
Increasing sodium absorption by overexpressing the epithelial sodium channel in mouse airways results in mucus accumulation and inflammation, changes that occur in the lungs of individuals with cystic fibrosis. The development of lung disease in these mice should provide insights into a disease that has long been lacking an animal model (pages 487-493).
引用
收藏
页码:452 / 454
页数:4
相关论文
共 11 条
[1]   Update on pathogenesis of cystic fibrosis lung disease [J].
Donaldson, SH ;
Boucher, RC .
CURRENT OPINION IN PULMONARY MEDICINE, 2003, 9 (06) :486-491
[2]   EXPRESSION OF THE CYSTIC-FIBROSIS GENE IN ADULT HUMAN LUNG [J].
ENGELHARDT, JF ;
ZEPEDA, M ;
COHN, JA ;
YANKASKAS, JR ;
WILSON, JM .
JOURNAL OF CLINICAL INVESTIGATION, 1994, 93 (02) :737-749
[3]   Binding of interleukin-8 to heparan sulfate and chondroitin sulfate in lung tissue [J].
Frevert, CW ;
Kinsella, MG ;
Vathanaprida, C ;
Goodman, RB ;
Baskin, DG ;
Proudfoot, A ;
Wells, TNC ;
Wight, TN ;
Martin, TR .
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY, 2003, 28 (04) :464-472
[4]  
Grubb Barbara R., 1999, Physiological Reviews, V79, pS193
[5]   EFFICIENCY OF GENE-TRANSFER FOR RESTORATION OF NORMAL AIRWAY EPITHELIAL FUNCTION IN CYSTIC-FIBROSIS [J].
JOHNSON, LG ;
OLSEN, JC ;
SARKADI, B ;
MOORE, KL ;
SWANSTROM, R ;
BOUCHER, RC .
NATURE GENETICS, 1992, 2 (01) :21-25
[6]   Matrilysin shedding of syndecan-1 regulates chemokine mobilization and transepithelial efflux of neutrophils in acute lung injury [J].
Li, QL ;
Park, PW ;
Wilson, CL ;
Parks, WC .
CELL, 2002, 111 (05) :635-646
[7]   Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice [J].
Mall, M ;
Grubb, BR ;
Harkema, JR ;
O'Neal, WK ;
Boucher, RC .
NATURE MEDICINE, 2004, 10 (05) :487-493
[8]   Plasminogen activator inhibitor-1 supports IL-8-mediated neutrophil transendothelial migration by inhibition of the constitutive shedding of endothelial IL-8/heparan sulfate/syndecan-1 complexes [J].
Marshall, LJ ;
Ramdin, LSP ;
Brooks, T ;
DPhil, PC ;
Shute, JK .
JOURNAL OF IMMUNOLOGY, 2003, 171 (04) :2057-2065
[9]   Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease [J].
Matsui, H ;
Grubb, BR ;
Tarran, R ;
Randell, SH ;
Gatzy, JT ;
Davis, CW ;
Boucher, RC .
CELL, 1998, 95 (07) :1005-1015
[10]  
Pilewski Joseph M., 1999, Physiological Reviews, V79, pS215