V-type proton ATPase (V-ATPase) is a proton pump that is highly conserved. It is a multi-subunit complex that consists of V0 domain responsible for the proton transport and V1 domain involved in the ATP hydrolysis. Each domain has multiple different subunits. The V0 domain includes 6 different subunits (a, d, c, c', c", and e). The V1 domain contains 8 subunits among which subunits D and F form a central rotor axle. Moreover, the V1 domain also contains tissue-specific subunit isoforms (B, C, E, and G). V-ATPase can hydrolyze ATP to drive a proton pump. It is found in the bio-membrane system, including endoplasmic reticulum, lysosomal membrane, plant tonoplasts, and the plasma membrane of prokaryotes. V-ATPase is involved in the various biological processes, such as pH homeostasis, receptor-mediated endocytosis, proteins transport, active transport of metabolites, neurotransmitter release, and tumor metastasis. Studies have shown that V-ATPase is associated with the progression of many diseases, such as osteopetrosis, distal renal tubular acidosis, X-linked myopathy with excessive autophagy.
Here shows part of V-ATPase in humans including ATP6V0A1, ATP6V0A2, ATP6V0A4, and ATP6V0C, encoded by ATP6V0A1, ATP6V0A2, ATP6V0A4, and ATP6V0C genes, respectively. They are distributed on the surface of the membrane of cell and organelle and act as the proton pumps to move protons across cell membranes involved in the multiple intracellular and extracellular processes.
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