Vergani, P; Gadsby, D; Csanády, L; (2012) CFTR, an Ion Channel Evolved from an ABC Transporter. In: Roberts, GK, (ed.) Encyclopedia of Biophysics. (254 - 265). Springer Berlin Heidelberg

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Abstract

CFTR (cystic fibrosis transmembrane conductance regulator) is an integral membrane protein that functions as an epithelial Cl- channel, which is rendered defective 2 by inherited gene mutations in patients with cystic fibrosis (Riordan et al. 1989). Its function underlies fluid secretion in the airways, sweat ducts, pancreatic duct, and vas deferens, and it mediates the excessive intestinal water loss in secretory diarrhoeas (Verkman and Galietta 2009). CFTR belongs to the large ABC (ATP-binding cassette) transporter superfamily, of which most members are ATP-hydrolyzing pumps. CFTR is the sole ABC protein known to function as an ion channel. Despite this difference in function, the gates of CFTR channels are opened and closed by interactions with ATP similar to those that in other ABC proteins result in energetically uphill substrate transport.

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