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ATP6V0A1 is a component of vacuolar ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of eukaryotic intracellular organelles. V-ATPase dependent organelle acidification is necessary for such intracellular processes as protein sorting, zymogen activation, receptor-mediated endocytosis, and synaptic vesicle proton gradient generation. V-ATPase is composed of a cytosolic V1 domain and a transmembrane V0 domain. The V1 domain consists of three A and three B subunits, two G subunits plus the C, D, E, F, and H subunits. The V1 domain contains the ATP catalytic site. The V0 domain consists of five different subunits: a, c, c', c", and d. Additional isoforms of many of the V1 and V0 subunit proteins are encoded by multiple genes or alternatively spliced transcript variants. This gene encodes one of three A subunit proteins and the encoded protein is associated with clathrin-coated vesicles. Three transcript variants encoding different isoforms have been found for this gene.
Gene Name: | ATPase, H+ transporting, lysosomal V0 subunit a1 |
Family/Subfamily: | Transporter , ATPase - V type |
Synonyms: | ATP6V0A1, ATP6N1, STV1, V-ATPase 116 kDa, VPP1, ATP6N1A, V-ATPase 116 kDa isoform a1, Vacuolar proton pump subunit 1, VPH1 |
Target Sequences: | NM_005177 NP_005168.2 Q93050 |
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