Nucleotide transport through the cystic fibrosis transmembrane conductance regulator.
The cystic fibrosis transmembrane conductance regulator (CFTR) is a member of the superfamily of ATP-binding cassette (ABC) transporters, also known as traffic ATPases, which are implicated in the movement of various substrates. Recent studies indicate that CFTR and other closely related ABC transporters are also implicated in the movement of cellular ATP. This is the subject of current controversy. Therefore, evidence for the movement of cellular nucleotides by expression of CFTR and related molecules, as well as the potential significance of ATP-permeable channels in cell physiology, are reviewed in this study. The hypothesis is thus forwarded for the improper delivery of cellular ATP to the extracellular milieu by a dysfunctional CFTR, to be a relevant factor in the onset of cystic fibrosis.
Cantiello
H F
HF
Renal Unit, Massachusetts General Hospital East, Charlestown 02129, USA.
D000255:Adenosine Triphosphate / Q000378:metabolism*; D000818:Animals; D001692:Biological Transport; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D006801:Humans
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D000255:Adenosine Triphosphate