抄録
<jats:p>Vacuolar H<jats:sup>+</jats:sup>-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<jats:sup>+</jats:sup>-ATPases fulfill special tasks in the kidney. Vacuolar H<jats:sup>+</jats:sup>-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<jats:sup>+</jats:sup>-ATPases are also found in endosomes, which are acidified by the pump. In addition, vacuolar H<jats:sup>+</jats:sup>-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<jats:sup>+</jats:sup>-ATPase in patients with distal renal tubular acidosis. The activity of vacuolar H<jats:sup>+</jats:sup>-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.</jats:p>
収録刊行物
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- Physiological Reviews
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Physiological Reviews 84 (4), 1263-1314, 2004-10
American Physiological Society
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詳細情報 詳細情報について
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- CRID
- 1360011145615989248
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- NII論文ID
- 30010383956
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- ISSN
- 15221210
- 00319333
- http://id.crossref.org/issn/00319333
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