V-type proton ATPase subunit B, kidney isoform (ATP6V1B1)

V-type proton ATPase subunit B, kidney isoform (ATP6V1B1) is the non-catalytic B1 subunit of the cytosolic V1 sector of vacuolar ATPase (V-ATPase), a multisubunit enzyme complex responsible for proton (H+) transport across intracellular organelle membranes and, in some cells, across the plasma membrane. This acidification is essential for processes such as urinary acidification in the kidney, protein sorting, endocytosis, synaptic vesicle function, and maintenance of pH in the inner ear. The B1 subunit is primarily expressed in the kidney and inner ear. Mutations in ATP6V1B1 disrupt normal acid-base homeostasis and are causative for distal renal tubular acidosis associated with progressive sensorineural hearing loss[1][2][3][7].

Other names

ATPase H+ transporting V1 subunit B1ATP6B1VATBVPP3V-ATPase subunit B1RTA1BVma2Endomembrane proton pump 58 kDa subunitVacuolar proton pump subunit B1Renal tubular acidosis with deafnessDRTA2H(+)-transporting two-sector ATPase 58kD subunitH+-ATPase beta 1 subunitvacuolar proton pump 3vacuolar proton pump subunit 3

Drugs targeting this enzyme would likely inhibit or modulate ATPase-mediated proton transport, interfering with organellar and urinary acidification, but clinically relevant inhibitors are not listed; no approved drugs known to target specifically ATP6V1B1 in humans[2][3][7].