V-type proton ATPase subunit B, kidney isoform; V-ATPase subunit B1; vacuolar proton pump subunit B1; endomembrane proton pump 58 kD subunit (ATP6V1B1, ATP6B1, VATB, VPP3)
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Function
- non-catalytic subunit of peripheral V1 complex of vacuolar ATPase
- forms a complex with SLC9A3R1 & SCL4A7
Structure
- PDZ-binding motif mediates interactions with SLC9A3R1 & SCL4A7
- belongs to the ATPase alpha/beta chains family
Compartment
- intracytoplasmic membrane
- peripheral membrane protein
- endomembrane
Expression
expressed in the cochlea & endolymphatic sac
Pathology
- defects in ATP6V1B1 are the cause of distal renal tubular acidosis with deafness