Source: Annual Congress 2009 - Cystic fibrosis: advances in clinical research
Disease area: Airway diseases

Abstract

In previous studies, the α-glucosidase inhibitor N-butyldeoxynojirimycin (miglustat) has been shown to restore cAMP-dependent chloride current in human nasal epithelial, tracheal gland serous, and pancreatic duct cell lines derived from DF508-homozygous CF-patients (Norez et al., FEBS Letters 580, 2006, 2081-2086). In the present study we have determined the effect of N-butyldeoxynojirimycin on chloride efflux from human bronchial epithelial cells, using the fluorescent probe MQAE.
Non-CF (16HBE) and CF (CFBE) bronchial epithelial cells were cultured under standard conditions in M199 medium at 37°C. The cells were exposed to 100 μM N-butyldeoxynojirimycin for 4 h and subsequently loaded with MQAE for 2 h.
After that, chloride efflux was determined from the increase of the MQAE fluorescence, when the cells were exposed to a solution in which all chloride was replaced by nitrate.
N-butyldeoxynojirimycin caused 126% increase in chloride efflux from CFBE cells (p=0.0059), in the absence of cAMP-elevating agents.
No significant effect of N-butyldeoxynojirimycin was observed in non-CF 16HBE cells, in which the chloride efflux in the absence of cAMP-elevating agents is about four times larger.
These results confirm that N-butyldeoxynojirimycin can partially correct the defective chloride efflux in CF airway epithelial cells.

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Citations should be made in the following way:
I. Oliynyk, G. M. Roomans (Örebro, Sweden). Effect of n-butyldeoxynojirimycin on chloride efflux from CF airway epithelial cells. Eur Respir J 2009; 34: Suppl. 53, 1263

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