Localization and regulation of the ATP6V0A4 (a4) vacuolar H+-ATPase subunit defective in an inherited form of distal renal tubular acidosis
PA Stehberger, N Schulz, KE Finberg… - Journal of the …, 2003 - journals.lww.com
Vacuolar-type H+-ATPases (VH+-ATPases) are the major H+-secreting protein in the distal
portion of the nephron and are involved in net H+ secretion (bicarbonate generation) or H+
reabsorption (net bicarbonate secretion). In addition, VH+-ATPases are involved in HCO 3−
reabsorption in the proximal tubule and distal tubule. VH+-ATPases consist of at least 13
subunits, the functions of which have not all been elucidated. Mutations in the accessory
ATP6V0A4 (a4 isoform) subunit have recently been shown to cause an inherited form of …
portion of the nephron and are involved in net H+ secretion (bicarbonate generation) or H+
reabsorption (net bicarbonate secretion). In addition, VH+-ATPases are involved in HCO 3−
reabsorption in the proximal tubule and distal tubule. VH+-ATPases consist of at least 13
subunits, the functions of which have not all been elucidated. Mutations in the accessory
ATP6V0A4 (a4 isoform) subunit have recently been shown to cause an inherited form of …