ATP6V1B2 (ATPase H+ Transporting V1 Subunit B2) is a Protein Coding gene. Diseases associated with ATP6V1B2 include Zimmermann-Laband Syndrome 2 and Deafness, Congenital, With Onychodystrophy, Autosomal Dominant. Among its related pathways are Rheumatoid arthritis and mTOR signaling pathway (KEGG). ATP6V1B2 encodes 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, three B, and two G subunits, as well as a C, D, E, F, and H subunit. The V1 domain contains the ATP catalytic site. The protein encoded by ATP6V1B2 is one of two V1 domain B subunit isoforms and is the only B isoform highly expressed in osteoclasts.