Ketoconazole activates Cl- conductance and blocks Cl- and fluid absorption by cultured cystic fibrosis (CFPAC-1) cells.
نویسندگان
چکیده
منابع مشابه
Selective activation of cystic fibrosis transmembrane conductance regulator Cl- and HCO3- conductances.
While cystic fibrosis transmembrane conductance regulator (CFTR) is well known to function as a Cl(-) channel, some mutations in the channel protein causing cystic fibrosis (CF) disrupt another vital physiological function, HCO(3)(-) transport. Pathological implications of derailed HCO(3)(-) transport are clearly demonstrated by the pancreatic destruction that accompany certain mutations in CF....
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The cystic fibrosis transmembrane conductance regulator (CFTR) Cl- channel belongs to the ATP-binding cassette (ABC) transporter superfamily and regulates Cl- secretion in epithelial cells for water secretion. Loss-of-function mutations to the CFTR gene cause dehydrated mucus on the apical side of epithelial cells and increase the susceptibility of bacterial infection, especially in the airway ...
متن کاملAirway surface fluid volume and Cl content in cystic fibrosis and normal bronchial xenografts.
We describe the use of an in vivo human bronchial xenograft model of cystic fibrosis (CF) and non-CF airways to investigate pathophysiological alterations in airway surface fluid (ASF) volume (Vs) and Cl content. Vs was calculated based on the dilution of an impermeable marker, [3H]inulin, during harvesting of ASF from xenografts with an isosmotic Cl-free solution. These calculations demonstrat...
متن کاملRegulation of Cl- channels in normal and cystic fibrosis airway epithelial cells by extracellular ATP.
The rate of Cl- secretion by human airway epithelium is determined, in part, by apical cell membrane Cl- conductance. In cystic fibrosis airway epithelia, defective regulation of Cl- conductance decreases the capability to secrete Cl-. Here we report that extracytosolic ATP in the luminal bath of cultured human airway epithelia increased transepithelial Cl- secretion and apical membrane Cl- per...
متن کاملVoltage-dependent Gating of the Cystic Fibrosis Transmembrane Conductance Regulator Cl− Channel
When excised inside-out membrane patches are bathed in symmetrical Cl--rich solutions, the current-voltage (I-V) relationship of macroscopic cystic fibrosis transmembrane conductance regulator (CFTR) Cl- currents inwardly rectifies at large positive voltages. To investigate the mechanism of inward rectification, we studied CFTR Cl- channels in excised inside-out membrane patches from cells expr...
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ژورنال
عنوان ژورنال: Proceedings of the National Academy of Sciences
سال: 1993
ISSN: 0027-8424,1091-6490
DOI: 10.1073/pnas.90.9.4047