ATP6V0A2 Part of the proton channel of V-ATPases. Essential component of the endosomal pH-sensing machinery. May play a role in maintaining the Golgi functions, such as glycosylation maturation, by controlling the Golgi pH. In aerobic conditions, involved in intracellular iron homeostasis, thus triggering the activity of Fe(2+) prolyl hydroxylase (PHD) enzymes, and leading to HIF1A hydroxylation and subsequent proteasomal degradation. Belongs to the V-ATPase 116 kDa subunit family. Note: This description may include information from UniProtKB.
Protein type: Energy Metabolism - oxidative phosphorylation; Membrane protein, integral; Membrane protein, multi-pass; Transporter; Transporter, ion channel; Transporter, iron
Chromosomal Location of Human Ortholog: 12q24.31
Cellular Component:  acrosomal vesicle; endosome membrane; integral component of membrane; perinuclear region of cytoplasm; phagocytic vesicle membrane; vacuolar proton-transporting V-type ATPase, V0 domain
Molecular Function:  protein binding
Biological Process:  ATP hydrolysis coupled proton transport; cellular iron ion homeostasis; cellular response to increased oxygen levels; immune response; insulin receptor signaling pathway; ion transmembrane transport; phagosome acidification; regulation of macroautophagy; transferrin transport
Disease: Cutis Laxa, Autosomal Recessive, Type Iia; Wrinkly Skin Syndrome
Reference #:  Q9Y487 (UniProtKB)
Alt. Names/Synonyms: A2; A2V-ATPase; ARCL; ARCL2A; ATP6A2; ATP6N1D; ATP6V0A2; ATPase H+ transporting V0 subunit a2; ATPase, H+ transporting, lysosomal V0 subunit a2; J6B7; Lysosomal H(+)-transporting ATPase V0 subunit a2; regeneration and tolerance factor; RTF; STV1; TJ6; TJ6M; TJ6S; v-ATPase 116 kDa; V-ATPase 116 kDa isoform a2; V-ATPase 116 kDa subunit a2; v-type proton ATPase 116 kDa subunit a; V-type proton ATPase 116 kDa subunit a isoform 2; V-type proton ATPase 116 kDa subunit a2; vacuolar proton translocating ATPase 116 kDa subunit a; Vacuolar proton translocating ATPase 116 kDa subunit a isoform 2; VPH1; VPP2; WSS
Gene Symbols: ATP6V0A2
Molecular weight: 98,082 Da
Basal Isoelectric point: 6.18  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene