Xenbase is experiencing difficulties due to technical problems with the University of Calgary IT infrastructure and may go temporarily offline.

Click on this message to dismiss it.
Click here to close Hello! We notice that you are using Internet Explorer, which is not supported by Xenbase and may cause the site to display incorrectly. We suggest using a current version of Chrome, FireFox, or Safari.
Novartis Found Symp. January 1, 2006; 273 177-86; discussion 186-92, 261-4.

Regulatory interaction between CFTR and the SLC26 transporters.

Shcheynikov N , Ko SB , Zeng W , Choi JY , Dorwart MR , Thomas PJ , Muallem S .

Most epithelia that express CFTR secrete fluid rich in HCO3- and poor in Cl- that is generated by a CFTR-dependent Cl- absorption and HCO3- secretion process that when aberrant leads to human diseases such as cystic fibrosis and congenital chloride diarrhoea. Epithelial Cl- absorption and HCO3- secretion require expression of CFTR and other Cl- and HCO3- transporters in the luminal membrane of the secreting cells. Recent advances in understanding this critical epithelial function revealed that the luminal Cl- and HCO3- transporters are members of the SLC26 family. Characterization of several members of the family reveals that all characterized thus far are electrogenic with an isoform specific Cl-/HCO3- transport stoichiometry. In vivo these transporters exist in a transporting complex with CFTR. The SLC26 transporters and CFTR are recruited to the complex by binding to scaffolds containing PDZ domains. Upon stimulation and PKA-dependent phosphorylation of CFTR R domain, the R domain binds to the SLC26 transporter STAS domain. Interaction of the R and STAS domains results in a marked and mutual activation of CFTR and the SLC26 transporters. The significance of this mode of regulation to epithelial Cl- absorption and HCO3- secretion is obvious.

PubMed ID: 17120768
Article link:

Genes referenced: cftr

My Xenbase: [ Log-in / Register ]
version: [3.11.2]

Major funding for Xenbase is provided by the National Institute of Child Health and Human Development, grant P41 HD064556