Relative ion permeability of normal and cystic fibrosis nasal epithelium.
نویسندگان
چکیده
منابع مشابه
Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium.
New treatments for cystic fibrosis (CF), including gene therapy, are currently being assessed. These aim to correct the basic defects of increased sodium absorption and decreased chloride secretion in airway epithelia. Assessment of these bioelectric parameters, particularly in the nasal epithelium, is likely to be used as a measure of treatment efficacy. However, the optimal in vivo protocol t...
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INTRODUCTION Although most individuals with cystic fibrosis (CF) develop progressive obstructive lung disease, disease severity is highly variable, even for individuals with similar CFTR mutations. Measurements of chloride transport as expression of CFTR function in nasal epithelial cells correlate with pulmonary function and suggest that F508del-CFTR is expressed at the apical membrane. Howeve...
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T he measurement of sweat electrolytes successfully diagnoses all but a tiny minority of patients with cystic fibrosis (CF). The discovery of a gene for CF, encoding the cystic fibrosis transmembrane conductance regulator (CFTR), has led to the understanding that CFTR is a chloride channel (hence the utility of the measurement of sweat electrolytes) and also regulates other ion channels, in par...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1983
ISSN: 0021-9738
DOI: 10.1172/jci110894