期刊
MOLECULAR BIOLOGY OF THE CELL
卷 16, 期 5, 页码 2154-2167出版社
AMER SOC CELL BIOLOGY
DOI: 10.1091/mbc.E04-11-1010
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- NHLBI NIH HHS [P01 HL034322, HL60280, HL34322, P50 HL060280] Funding Source: Medline
- NIDDK NIH HHS [DK51870, R01 DK051870] Funding Source: Medline
Previous studies in native tissues have produced conflicting data on the localization and metabolic fate of WT and Delta F508 cystic fibrosis transmembrane regulator (CFTR) in the lung. Combining immunocytochemical and biochemical studies utilizing new high-affinity CFTR mAbs with ion transport assays, we examined both 1) the cell type and region specific expression of CFTR in normal airways and 2) the metabolic fate of Delta F508 CFTR and associated ERM proteins in the cystic fibrosis lung. Studies of lungs from a large number of normal subjects revealed that WT CFTR protein localized to the apical membrane of ciliated cells within the superficial epithelium and gland ducts. In contrast, other cell types in the superficial, gland acinar, and alveolar epithelia expressed little WT CFTR protein. No AF508 CFTR mature protein or function could be detected in airway specimens freshly excised from a large number of AF508 homozygous subjects, despite an intact ERM complex. In sum, our data demonstrate that WT CFTR is predominantly expressed in ciliated cells, and Delta F508 CFTR pathogenesis in native tissues, like heterologous cells, reflects loss of normal protein processing.
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