V-type proton ATPase catalytic subunit A (ATP6V1A)

V-type proton ATPase catalytic subunit A (ATP6V1A) is a ubiquitous enzyme subunit that forms part of the V1 domain of the vacuolar-type H+-ATPase (V-ATPase) complex. V-ATPases are responsible for the ATP-dependent acidification of intracellular organelles, which is crucial for a variety of processes including protein sorting, receptor-mediated endocytosis, synaptic vesicle loading, and lysosomal degradation. The A subunit is the catalytic core, directly involved in ATP hydrolysis, providing the energy for proton transport by the V0 domain across organelle membranes. Mutations in ATP6V1A can lead to disorders such as developmental and epileptic encephalopathy and connective tissue diseases. Pharmacological inhibition of V-ATPase, such as by bafilomycin or concanamycin, is a useful research tool but highly toxic due to the central role of ATP6V1A-containing complexes in cellular physiology[1][2][5].

Other names

ATPase H+ transporting V1 subunit AATP6A1ATP6V1A1VPP2Vma1VA68ARCL2DDEE93HO68IECEE3

Inhibition of proton transport by blocking ATP hydrolysis (V-ATPase inhibitors prevent acidification of intracellular compartments)