ATP6V1H

V-type proton ATPase subunit H is an is_associated_with::enzyme that in humans is encoded by the ATP6V1H is_associated_with::gene.

Function
This gene encodes a component of is_associated_with::vacuolar is_associated_with::ATPase (V-ATPase), a multisubunit enzyme that mediates acidification of is_associated_with::eukaryotic intracellular is_associated_with::organelles. V-ATPase dependent organelle acidification is necessary for such intracellular processes as protein sorting, is_associated_with::zymogen activation, receptor-mediated is_associated_with::endocytosis, and is_associated_with::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 the regulatory H subunit of the V1 domain which is required for catalysis of ATP but not the assembly of V-ATPase. Three alternatively spliced transcript variants encode two isoforms of the H subunit.