ATP6V1G3


Description

The ATP6V1G3 (ATPase H+ transporting V1 subunit G3) is a protein-coding gene located on chromosome 1.

ATP6V1G3 gene encodes V-type proton ATPase subunit G 3, an enzyme involved in acidifying eukaryotic intracellular organelles. This enzyme is part of vacuolar ATPase (V-ATPase), a multi-subunit complex responsible for crucial intracellular processes like protein sorting, zymogen activation, receptor-mediated endocytosis, and synaptic vesicle proton gradient generation. V-ATPase is composed of two domains: the cytosolic V1 domain and the transmembrane V0 domain. The V1 domain consists of three A and three B subunits, two G subunits, and other subunits like C, D, E, F, and H. The V1 domain houses the ATP catalytic site. The V0 domain includes five subunits: a, c, c', c'' and d. Multiple genes and alternatively spliced transcript variants encode isoforms of V1 and V0 subunits. ATP6V1G3 encodes one of three G subunit proteins, with transcript variants encoding different isoforms.

ATP6V1G3 encodes a subunit of the V1 complex of vacuolar (H+)-ATPase (V-ATPase), a multi-subunit enzyme that hydrolyzes ATP to power proton translocation across membranes. V-ATPase is critical for maintaining the acidic pH of intracellular compartments like lysosomes and endosomes. In some cell types, it can also be targeted to the plasma membrane, where it contributes to acidifying the extracellular environment.

ATP6V1G3 is also known as ATP6G3, Vma10.

Associated Diseases



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