Journal
PHYSIOLOGICAL REVIEWS
Volume 84, Issue 4, Pages 1263-1314Publisher
AMER PHYSIOLOGICAL SOC
DOI: 10.1152/physrev.00045.2003
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Funding
- NICHD NIH HHS [HD 40793] Funding Source: Medline
- NIDDK NIH HHS [DK 38452, DK 17433, DK 50230, DK 42595, DK 32573] Funding Source: Medline
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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 organelles, vacuolar H+-ATPases fulfill special tasks in the kidney. Vacuolar H+-ATPases are expressed in the plasma membrane in the kidney almost along the entire length of the nephron with apical and/or basolateral localization patterns. In the proximal tubule, a high number of vacuolar H+-ATPases are also found in endosomes, which are acidified by the pump. In addition, vacuolar H+-ATPases contribute to proximal tubular bicarbonate reabsorption. The importance in final urinary acidification along the collecting system is highlighted by monogenic defects in two subunits (ATP6V0A4, ATP6V1B1) of the vacuolar H+-ATPase in patients with distal renal tubular acidosis. The activity of vacuolar H+-ATPases is tightly regulated by a variety of factors such as the acid-base or electrolyte status. This regulation is at least in part mediated by various hormones and protein-protein interactions between regulatory proteins and multiple subunits of the pump.
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