PMID: 20051483

Kelly M, Trudel S, Brouillard F, Bouillaud F, Colas J, Nguyen-Khoa T, Ollero M, Edelman A, Fritsch J
Cystic fibrosis transmembrane regulator inhibitors CFTR(inh)-172 and GlyH-101 target mitochondrial functions, independently of chloride channel inhibition.
J Pharmacol Exp Ther. 2010 Apr;333(1):60-9. Epub 2010 Jan 5., [PubMed]
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No. Mutations Sentence Comment
3 ABCC7 p.Trp1282*
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ABCC7 p.Trp1282* 20051483:3:260
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 In the present study, we investigated the effects of the two compounds on reactive oxygen species (ROS) production and mitochondrial membrane potential in several cell lines: the CFTR-deficient human lung epithelial IB3-1 (expressing the heterozygous F508del/ W1282X mutation), the isogenic CFTR-corrected C38, and HeLa and A549 as non-CFTR-expressing controls. Login to comment 49 ABCC7 p.Trp1282*
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ABCC7 p.Trp1282* 20051483:49:70
status: NEW
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 The IB3-1 human CF bronchial epithelial cell line (mutations F508del/ W1282X) and the stably rescued cell line C38 expressing CFTR were grown on fibronectin-coated culture flasks in Laboratory of Human Carcinogenesis basal medium 8 supplemented with 1% heat-inactivated fetal calf serum (FCS). Login to comment