BACKGROUND: Channelopathies, defined as diseases that are caused by mutations in
genes encoding ion channels, are associated with a wide variety of symptoms and have …

Vacuolar H+-ATPases are ubiquitous multisubunit complexes mediating the ATP-dependent
transport of protons. In addition to their role in acidifying the lumen of various intracellular …

Nuclear receptors are transcription factors that regulate diverse cellular processes. In
canonical activation, ligand availability is sufficient to produce receptor binding, entraining …

A number of ion channels and transporters are expressed in both the inner ear and kidney.
In the inner ear, K+ cycling and endolymphatic K+, Na+, Ca2+, and pH homeostasis are …

Cystic fibrosis (CF) is caused by defects in an anion channel, the cystic fibrosis
transmembrane conductance regulator (CFTR). Recently, a new airway epithelial cell type …

Pendrin is an anion exchanger expressed along the apical plasma membrane and apical
cytoplasmic vesicles of type B and of non-A, non-B intercalated cells of the distal convoluted …

The family of mammalian bicarbonate transport proteins are involved in a wide-range of
physiological processes. The importance of bicarbonate transport follows from the …

Slc26a4 (Pds, pendrin) is an anion transporter expressed in the apical region of type B and
non-A, non-B intercalated cells of the distal nephron. It is upregulated by aldosterone …

Chronic metabolic acidosis results in renal Ca 2+ and Mg 2+ wasting, whereas chronic
metabolic alkalosis is known to exert the reverse effects. It was hypothesized that these …

The renal collecting system serves the fine-tuning of renal acid–base secretion. Acid-
secretory type-A intercalated cells secrete protons via a luminally expressed V-type H+ …