Normal wtCFTR traffic/ ER-to-Golgi
The cystic fibrosis transmembrane conductance regulator ( CFTR ) is a member of the ATP-binding cassette transporter superfamily. It acts in apical part of the epithelial cells as a plasma-membrane cyclic AMP-activated chloride anion, bicarbonate anion and glutathione channel , , . Cell surface expression of the CFTR is a highly regulated intracellular process , .
A G olgi associated PDZ and coiled-coil motif containing ( PIST ) regulates CFTR trafficking. PIST causes a reduction in the number of CFTR channels in the plasma membrane and facilitates trafficking of CFTR to lysosomes , , . PIST action is activated by Syntaxin 6 , (Cheng et al., The 21st annual north American cystic fibrosis conference, California, 2007 ) and is inhibited by R as homolog gene family, member Q ( TC10 ) .
CFTR modified in ER and/or Golgi may be delivered from the Golgi to the apical membrane, possibly, with participation of coat protein complex Coatomer .