V-type proton ATPase subunit H (ATP6V1H)

V-type proton ATPase subunit H (ATP6V1H) is a pivotal regulatory subunit of the V1 complex in vacuolar ATPases (V-ATPase), enzymes essential for acidifying various intracellular organelles in eukaryotic cells, including endosomes, lysosomes, and synaptic vesicles. Proper acidification mediated by V-ATPase is critical for processes such as protein sorting, activation of precursor enzymes (zymogens), formation of proton gradients, and receptor-mediated endocytosis. The subunit H is vital for the enzyme’s activity, though not required for its assembly, and defects or alterations in its function are linked to disorders such as lateral meningocele syndrome, osteogenesis imperfecta type V, and may contribute to metabolic diseases including type 2 diabetes. Drugs such as tiludronic acid may act as V-ATPase inhibitors, although their pharmacological details regarding ATP6V1H are not comprehensively established.

Other names

NBP1Nef-binding protein 1Protein VMA13 homologV-ATPase 50/57 kDa subunitsV-ATPase subunit HVacuolar proton pump subunit HVacuolar proton pump subunit SFDSFDSFDalphaSFDbetaVMA13CGI-11

Inhibition of V-ATPase proton transport