Target intelligence / Profile preview

V-type proton ATPase subunit C 2 (ATP6V1C2)

Target
ATP6V1C2
Molecular classification
Enzyme, Transporter, Subunit of vacuolar ATPase (V-ATPase)
01

Overview

V-type proton ATPase subunit C 2 (ATP6V1C2) is a component of the cytosolic V1 domain of vacuolar ATPase (V-ATPase), a multisubunit enzyme that acidifies intracellular organelles such as lysosomes, endosomes, and secretory vesicles in eukaryotic cells[1][3][5]. The V1 domain hydrolyzes ATP, providing the energy to drive proton transport via the membrane V0 domain; subunit C acts as a flexible stator, stabilizing the interface between the catalytic and membrane sectors and is necessary for assembly and activity regulation of the complex[2][4][8]. V-ATPase-dependent acidification is essential for key cellular processes including protein degradation, membrane trafficking, receptor-mediated endocytosis, and neurotransmitter loading into synaptic vesicles[1][3][6]. Dysfunction of V-ATPase subunits, including ATP6V1C2, has been linked to diseases such as renal tubular acidosis and may affect other pathologies related to defective organelle acidification[3][6].

Other names
ATPase H+ transporting V1 subunit C2V-ATPase subunit C2VMA5ATP6C2Vacuolar proton pump subunit C2VATC2
02

Mechanism of action

Inhibition of proton transport and organelle acidification by blocking V-ATPase function

03

Biological functions

Intracellular organelle acidificationProtein sortingZymogen activationReceptor-mediated endocytosisSynaptic vesicle proton gradient generationPositive regulation of Wnt signaling pathway[7]
04

Disease associations

Distal renal tubular acidosis[3]Renal tubular acidosis, distal, type 1[3]Potential implication in other diseases associated with lysosomal function and acidification[6]
05

Safety considerations

Potential disruption of lysosomal and vesicular pH homeostasisBroad inhibition of V-ATPase enzymes influences multiple cellular processes, possibly leading to cytotoxicity[6]
06

Interacting drugs

Bafilomycin A1 (V-ATPase general inhibitor, not selective for subunit C2)

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