Predicted to enable ATPase binding activity. Involved in nematode larval development. Located in apical plasma membrane. Part of vacuolar proton-transporting V-type ATPase complex. Is expressed in several structures, including Psub2; excretory cell; intestine; pharyngeal-intestinal valve; and uterus. Human ortholog(s) of this gene implicated in autosomal recessive cutis laxa type IIA; autosomal recessive osteopetrosis 1; developmental and epileptic encephalopathy 104; renal tubular acidosis; and wrinkly skin syndrome. Orthologous to several human genes including ATP6V0A1 (ATPase H+ transporting V0 subunit a1); ATP6V0A2 (ATPase H+ transporting V0 subunit a2); and ATP6V0A4 (ATPase H+ transporting V0 subunit a4).
WB Description
vha-6 encodes an ortholog of subunit a of the membrane-bound (V0) domainof vacuolar proton-translocating ATPase (V-ATPase); VHA-6 is orthologousto human ATP6N1A (OMIM:192130), ATP6V0A2, ATP6V0A4 (OMIM:605239, mutatedin distal renal tubular acidosis), and TCIRG1 (OMIM:604592, mutated inosteopetrosis); VHA-6 is expressed in germline (P2 through Z3 cellsuntil the comma stage), on the apical surface of postembryonicintestinal cells, and at the junction between pharynx and intestine;VHA-6 is required for growth and survival past the L1 larval stage;vha-6(RNAi) animals arrest as L1 larvae and remain alive (but do notgrow) for one week; in S. cerevisiae, different V0 a-subunits (Stv1p andVph1p) direct the assembly of V-ATPases to different membranes andorganelles, suggesting that the profusion of such subunits in C. elegans(co-orthologous VHA-5, VHA-6, VHA-7, and six UNC-32 isoforms) may have asimilar function; VHA-6 is predicted to capture protons from V-ATPasetransmembrane rotor components and export the protons across themembrane.