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CFTR, an Ion Channel Evolved from an ABC Transporter

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 Green open access

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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.

Type: Book chapter
Title: CFTR, an Ion Channel Evolved from an ABC Transporter
ISBN-13: 978-3-642-16711-9
Open access status: An open access version is available from UCL Discovery
DOI: 10.1007/978-3-642-16712-6_364
Language: English
Additional information: © European Biophysical Societies’ Association (EBSA). The final publication is available at http://www.springerreference.com/docs/html/chapterdbid/332458.html
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Life Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Life Sciences > Div of Biosciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Life Sciences > Div of Biosciences > Neuro, Physiology and Pharmacology
URI: https://discovery.ucl.ac.uk/id/eprint/1403738
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