TY - JOUR
T1 - Kidney Vacuolar H+-ATPase
T2 - Physiology and Regulation
AU - Valles, Patricia
AU - Lapointe, Michael S.
AU - Wysocki, Jan
AU - Batlle, Daniel
PY - 2006/9/1
Y1 - 2006/9/1
N2 - The vacuolar H+-ATPase is a multisubunit protein consisting of a peripheral catalytic domain (V1) that binds and hydrolyzes adenosine triphosphate (ATP) and provides energy to pump H+ through the transmembrane domain (V0) against a large gradient. This proton-translocating vacuolar H+-ATPase is present in both intracellular compartments and the plasma membrane of eukaryotic cells. Mutations in genes encoding kidney intercalated cell-specific V0 a4 and V1 B1 subunits of the vacuolar H+-ATPase cause the syndrome of distal tubular renal acidosis. This review focuses on the function, regulation, and the role of vacuolar H+-ATPases in renal physiology. The localization of vacuolar H+-ATPases in the kidney, and their role in intracellular pH (pHi) regulation, transepithelial proton transport, and acid-base homeostasis are discussed.
AB - The vacuolar H+-ATPase is a multisubunit protein consisting of a peripheral catalytic domain (V1) that binds and hydrolyzes adenosine triphosphate (ATP) and provides energy to pump H+ through the transmembrane domain (V0) against a large gradient. This proton-translocating vacuolar H+-ATPase is present in both intracellular compartments and the plasma membrane of eukaryotic cells. Mutations in genes encoding kidney intercalated cell-specific V0 a4 and V1 B1 subunits of the vacuolar H+-ATPase cause the syndrome of distal tubular renal acidosis. This review focuses on the function, regulation, and the role of vacuolar H+-ATPases in renal physiology. The localization of vacuolar H+-ATPases in the kidney, and their role in intracellular pH (pHi) regulation, transepithelial proton transport, and acid-base homeostasis are discussed.
KW - ATP6V0A4 subunit
KW - ATP6V1B1 subunit
KW - distal tubular renal acidosis (dRTA)
KW - renal vacuolar H-ATPase
UR - http://www.scopus.com/inward/record.url?scp=33751007867&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33751007867&partnerID=8YFLogxK
U2 - 10.1016/j.semnephrol.2006.07.004
DO - 10.1016/j.semnephrol.2006.07.004
M3 - Article
C2 - 17071330
AN - SCOPUS:33751007867
SN - 0270-9295
VL - 26
SP - 361
EP - 374
JO - Seminars in nephrology
JF - Seminars in nephrology
IS - 5
ER -