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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.
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| 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 |
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Cellular Component:
acrosomal vesicle; endosome membrane; focal adhesion; intracellular membrane-bounded organelle; perinuclear region of cytoplasm; phagocytic vesicle membrane; plasma membrane; proton-transporting V-type ATPase complex; transmembrane transporter complex; vacuolar proton-transporting V-type ATPase complex; vacuolar proton-transporting V-type ATPase, V0 domain
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Molecular Function:
ATPase binding; protein binding; proton-transporting ATPase activity, rotational mechanism
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Biological Process:
cellular response to increased oxygen levels; Golgi lumen acidification; immune response; intracellular iron ion homeostasis; proton transmembrane transport; regulation of macroautophagy; vacuolar acidification
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Disease: Cutis Laxa, Autosomal Recessive, Type Iia; Wrinkly Skin Syndrome
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Reference #:
Q9Y487
(UniProtKB)
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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 2; 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
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Gene Symbols: ATP6V0A2
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Molecular weight:
98,082 Da
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Basal Isoelectric point:
6.18
Predict pI for various phosphorylation states
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