SUBMUCOSAL GLANDS ARE THE PREDOMINANT SITE OF CFTR EXPRESSION IN THE HUMAN BRONCHUS

被引：553
作者：
ENGELHARDT, JF
YANKASKAS, JR
ERNST, SA
YANG, YP
MARINO, CR
BOUCHER, RC
COHN, JA
WILSON, JM
机构：
[1] UNIV MICHIGAN,HOWARD HUGHES MED INST,ANN ARBOR,MI 48109
[2] UNIV MICHIGAN,DEPT INTERNAL MED,ANN ARBOR,MI 48109
[3] UNIV MICHIGAN,DEPT BIOL CHEM,ANN ARBOR,MI 48109
[4] UNIV MICHIGAN,DEPT ANAT & CELL BIOL,ANN ARBOR,MI 48109
[5] UNIV N CAROLINA,DIV PULM DIS,CHAPEL HILL,NC 27599
[6] YALE UNIV,DEPT INTERNAL MED,NEW HAVEN,CT 06510
[7] DUKE UNIV,DEPT MED,DURHAM,NC 27706
[8] VET ADM MED CTR,DURHAM,NC 27705
来源：
NATURE GENETICS | 1992年 / 2卷 / 03期
关键词：
D O I：
10.1038/ng1192-240
中图分类号：
Q3 [遗传学];
学科分类号：
071007 ; 090102 ;
摘要：
We have used in situ hybridization and immunocytochemistry to characterize the cellular distribution of cystic fibrosis (CF) gene expression in human bronchus. The cystic fibrosis transmembrane conductance regulator (CFTR) was primarily localized to cells of submucosal glands in bronchial tissues from non-CF individuals notably in the serous component of the secretory tubules as well as a subpopulation of cells in ducts. Normal distribution of CFTR mRNA was found in CF tissues while expression of CFTR protein was genotype specific, with AF508 homozygotes demonstrating no detectable protein and compound heterozygotes expressing decreased levels of normally distributed protein. Our data suggest mechanisms whereby defects in CFTR expression could lead to abnormal production of mucus in human lung.
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页码：240 / 248
页数：9
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