Document Detail


The CFTR ion channel: gating, regulation, and anion permeation.
MedLine Citation:
PMID:  23284076     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Cystic fibrosis transmembrane conductance regulator (CFTR) is an ATP-gated anion channel with two remarkable distinctions. First, it is the only ATP-binding cassette (ABC) transporter that is known to be an ion channel--almost all others function as transport ATPases. Second, CFTR is the only ligand-gated channel that consumes its ligand (ATP) during the gating cycle--a consequence of its enzymatic activity as an ABC transporter. We discuss these special properties of CFTR in the context of its evolutionary history as an ABC transporter. Other topics include the mechanisms by which CFTR gating is regulated by phosphorylation of its unique regulatory domain and our current view of the CFTR permeation pathway (or pore). Understanding these basic operating principles of the CFTR channel is central to defining the mechanisms of action of prospective cystic fibrosis drugs and to the development of new, rational treatment strategies.
Authors:
Tzyh-Chang Hwang; Kevin L Kirk
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural     Date:  2013-01-01
Journal Detail:
Title:  Cold Spring Harbor perspectives in medicine     Volume:  3     ISSN:  2157-1422     ISO Abbreviation:  Cold Spring Harb Perspect Med     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-01-03     Completed Date:  2013-07-22     Revised Date:  2013-11-14    
Medline Journal Info:
Nlm Unique ID:  101571139     Medline TA:  Cold Spring Harb Perspect Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  a009498     Citation Subset:  IM    
Affiliation:
Dalton Cardiovascular Research Center, University of Missouri-Columbia, Columbia, MO 65211, USA.
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MeSH Terms
Descriptor/Qualifier:
ATP-Binding Cassette Transporters / genetics
Adenosine Triphosphate / metabolism
Anions / metabolism
Cystic Fibrosis / drug therapy,  genetics*,  metabolism
Cystic Fibrosis Transmembrane Conductance Regulator / genetics*
Humans
Ion Channel Gating / genetics*
Ion Channels / metabolism
Ligand-Gated Ion Channels / genetics
Phosphorylation / genetics
Grant Support
ID/Acronym/Agency:
R01 DK056796/DK/NIDDK NIH HHS; R01 DK056796/DK/NIDDK NIH HHS; R01 DK55835/DK/NIDDK NIH HHS; R01 HL058341/HL/NHLBI NIH HHS; R01 HL53445/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Anions; 0/CFTR protein, human; 0/Ion Channels; 0/Ligand-Gated Ion Channels; 126880-72-6/Cystic Fibrosis Transmembrane Conductance Regulator; 56-65-5/Adenosine Triphosphate

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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