ATPase H+ transporting accessory protein 2 (ATP6AP2)

ATPase H+ transporting accessory protein 2 is a multifunctional protein serving as an accessory subunit to the V-type ATPase proton pump complex and as a cellular receptor for renin and prorenin. It is central to the acidification and maintenance of endo-lysosomal pH critical for protein degradation, cell signaling, and organelle function. As the prorenin/renin receptor, it mediates intracellular signaling cascades (notably involving ERK1/2) and increases the efficiency of angiotensinogen conversion to angiotensin I, linking it to the renin-angiotensin system and blood pressure regulation. Genetic mutations and dysregulation of its function are associated with neurodevelopmental and metabolic disorders, making it both a valuable diagnostic biomarker and a potential therapeutic target.

Other names

Renin receptor (PRR)Prorenin receptorRENRATP6M8-9ATP6IP2Vacuolar ATP synthase membrane sector-associated protein M8-9ATPase, H+ transporting, lysosomal interacting protein 2ER-Localized Type I Transmembrane AdapterEmbryonic Liver Differentiation Factor 10 (ELDF10)M8-9Vacuolar proton ATP synthase membrane sector associated protein M8-9ENSEMBL: ENSG00000182220UniProt: O75787

Drugs would act by inhibition/activation of receptor-mediated signaling (renin/prorenin pathway) Modulation of V-ATPase assembly and lysosomal acidification Blockage of ERK1/2 downstream signaling