Cystic fibrosis transmembrane conductance regulator (CFTR) is an ABC (ATP-binding cassette) transporter-class ion channel that transports chloride and thiocyanate ions across epithelial cell membranes[1]. Like the P2X receptors, the CFTR has an ATP binding domain, and is considered a cAMP-activated ATP-gated anion channel. It is found in the epithelial cells of many organs including the lung, liver, pancreas, digestive tract, reproductive tract, and skin. Chloride transport through the CFTR channel works in concert with sodium transport through epithelial sodium channels (ENaC) to maintain salt, fluid, and pH homeostasis in various epithelial tissues. Mutations of the CFTR gene affect functioning of the chloride ion channels in these cell membranes, leading to cystic fibrosis and congenital absence of the vas deferens[2].

[1] The ABC protein turned chloride channel whose failure causes cystic fibrosis. D.C. Gadsby, P. Vergani,  L. Csanády. Nature 2006, 440, 477-483.
[2] Cystic fibrosis transmembrane regulator protein mutations: 'class' opportunity for novel drug innovation. K.D. MacDonald, K.R. McKenzie, P.L.Zeitlin. Paediatr. Drugs 2007, 9, 1-10.

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Axon ID Name Description From price
2552 Adjudin Non-hormonal male contraceptive with anti-proliferative activity €125.00
4049 Ataluren CFTR-G542X suppressor €110.00
1763 CoPo 22 Modulator of CFTR (δ-F508 Selective) €120.00
2572 GlyH 101 Highly potent and selective CFTR inhibitor €105.00
3234 Lumacaftor Selective and orally bioavailable CFTR corrector €70.00
2295 PPQ 102 CFTR inhibitor that acts by a mechanism involving stabilization of the channel closed-state €100.00
2169 VX 661 Corrector of the cystic fibrosis transmembrane conductance regulator (CFTR) €80.00
2503 VX 770 Orally bioavailable CFTR potentiator €125.00

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