V-type proton ATPase subunit E 2 (ATP6V1E2)

V-type proton ATPase subunit E 2 (ATP6V1E2) is a component of the peripheral V1 domain of the vacuolar H^+-ATPase (V-ATPase), a multi-subunit enzyme complex involved in acidification of eukaryotic intracellular organelles such as lysosomes, endosomes, and secretory vesicles. The V1 domain is responsible for ATP hydrolysis, which powers the rotation of the central stalk and triggers proton translocation by the membrane-embedded V0 domain. The E2 subunit is predicted to enable proton-transporting ATPase activity and is necessary for maintaining the acidic environment required for protein degradation, autophagy, and cellular signaling processes. Mutations or dysfunction in V-ATPase subunits, including ATP6V1E2, are implicated in neurological and developmental disorders, lysosomal storage diseases, and renal defects. V-ATPase is considered a therapeutic target in conditions where modulation of organellar pH or autophagy is desirable, but systemic inhibition presents significant safety concerns due to its ubiquitous essential functions.

Other names

VATE2ATP6E1ATP6EL2ATP6V1EL2V-ATPase subunit E 2MGC9341VMA4Vacuolar proton pump subunit E 2Testis secretory sperm-binding protein Li 235PVacuolar-type proton-translocating ATPase subunit E1Lysosomal 31kDa V1 subunit E2V-ATPase subunit E2

Inhibition of proton translocation by blocking V-ATPase, leading to elevated lysosomal pH and suppression of autophagy; Inhibition of ATP-driven proton transport