AAH22300.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
98,082 Da
NCBI Official Full Name
ATP6V0A2 protein
NCBI Official Synonym Full Names
ATPase H+ transporting V0 subunit a2
NCBI Official Synonym Symbols
A2; RTF; TJ6; WSS; ARCL; J6B7; STV1; TJ6M; TJ6S; VPH1; ARCL2A; ATP6A2; ATP6N1D [Similar Products]
NCBI Protein Information
V-type proton ATPase 116 kDa subunit a isoform 2; V-type proton ATPase 116 kDa subunit a
UniProt Protein Name
V-type proton ATPase 116 kDa subunit a isoform 2
UniProt Synonym Protein Names
Lysosomal H(+)-transporting ATPase V0 subunit a2; TJ6; Vacuolar proton translocating ATPase 116 kDa subunit a isoform 2
UniProt Synonym Gene Names
NCBI Summary for ATP6V0A2
The protein encoded by this gene is a subunit of the vacuolar ATPase (v-ATPase), an heteromultimeric enzyme that is present in intracellular vesicles and in the plasma membrane of specialized cells, and which is essential for the acidification of diverse cellular components. V-ATPase is comprised of a membrane peripheral V(1) domain for ATP hydrolysis, and an integral membrane V(0) domain for proton translocation. The subunit encoded by this gene is a component of the V(0) domain. Mutations in this gene are a cause of both cutis laxa type II and wrinkly skin syndrome. [provided by RefSeq, Jul 2009]
UniProt Comments for 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 Fe2+ prolyl hydroxylase (PHD) enzymes, and leading to HIF1A hydroxylation and subsequent proteasomal degradation (PubMed:28296633).
Product References and Citations for anti-ATP6V0A2 antibody
(1) Fischer, B., Dimopoulou, A., Egerer, J., et al. Further characterization of ATP6V0A2-related autosomal recessive cutis laxa. Hum. Genet. 131 (11), 1761-1773 (2012).
Research Articles on ATP6V0A2
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Products associated with anti-ATP6V0A2 antibody
Pathways associated with anti-ATP6V0A2 antibody
Diseases associated with anti-ATP6V0A2 antibody
Organs/Tissues associated with anti-ATP6V0A2 antibody
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