Nucleotide transport through the cystic fibrosis transmembrane conductance regulator

CANTIELLO, HORACIO F.

BIOSCIENCE REPORTS

PORTLAND PRESS LTD

Año: 1997 vol. 17 p. 147 - 171

0144-8463

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.