Knowledge regarding this important system of renal chloride transporters has rapidly accumulated. A severe salt-losing tubulopathy—Bartter syndrome type III—develops when ClCKB is non-functional, whereas a common genetic variant of theCLCNKBgene results in salt-dependent hypertension. Disruption of the Barttin gene manifests as Bartter syndrome type IV with sensorineural deafness and an especially severe salt-losing phenotype. It is timely, therefore, to review the properties of these transporters.
- Bernhard K Krämer
- Tobias Bergler
- Siegfried Waldegger
