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Int J Biochem Cell Biol. 2014 Jul;52:15-25. doi: 10.1016/j.biocel.2014.02.004. Epub 2014 Feb 15.

CFTR structure and cystic fibrosis.

Author information

1
University of Manchester, Faculty of Life Sciences, Michael Smith Building, Manchester M13 9PT, UK.
2
University of Manchester, Faculty of Life Sciences, Michael Smith Building, Manchester M13 9PT, UK. Electronic address: robert.ford@manchester.ac.uk.

Abstract

CFTR (cystic fibrosis transmembrane conductance regulator) is a member of the ATP-binding cassette family of membrane proteins. Although almost all members of this family are transporters, CFTR functions as a channel with specificity for anions, in particular chloride and bicarbonate. In this review we look at what is known about CFTR structure and function within the context of the ATP-binding cassette family. We also review current strategies aimed at obtaining the high resolution structure of the protein.

KEYWORDS:

CFTR; Cystic fibrosis; Ion channel; Membrane protein; Protein structure

PMID:
24534272
DOI:
10.1016/j.biocel.2014.02.004
[Indexed for MEDLINE]

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