V-ATPase a1 Antibodies

Target Information

V-ATPase a1 (encoded by ATP6V0A1) is an integral membrane subunit of the vacuolar-type H+-ATPase (V-ATPase) that localizes predominantly to intracellular membranes, including endosomes and lysosomes, and is also enriched in neuronal compartments such as synaptic vesicles/clathrin-coated vesicles where it supports neurotransmitter loading. Structurally, V-ATPase is organized into a peripheral V1 ATP-hydrolytic sector and a membrane-embedded V0 proton-translocation sector; a1 is the V0 "a" isoform that provides a large N-terminal cytosolic domain important for V1-V0 assembly/regulation and a C-terminal multi-pass transmembrane region that interfaces with the proteolipid ring to enable proton movement across the membrane. Functionally, a1-containing V-ATPase complexes use ATP hydrolysis to pump protons into organelle lumens, generating acidity and electrochemical gradients required for protein sorting and degradation, receptor-mediated endocytosis, and vesicular trafficking, and in neurons specifically to establish the proton motive force that drives vesicular neurotransmitter uptake; consistent with this role, disease-associated ATP6V0A1 variants impair lysosomal acidification.

Synonyms

T17D12.8; T17D12_8; VHA-A1