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Identification
HMDB Protein ID HMDBP03006
Secondary Accession Numbers
  • 8528
Name V-type proton ATPase 116 kDa subunit a isoform 2
Synonyms
  1. Lysosomal H(+)-transporting ATPase V0 subunit a2
  2. TJ6
  3. V-ATPase 116 kDa isoform a2
  4. Vacuolar proton translocating ATPase 116 kDa subunit a isoform 2
Gene Name ATP6V0A2
Protein Type Enzyme
Biological Properties
General Function Energy production and conversion
Specific Function 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
Pathways Not Available
Reactions Not Available
GO Classification
Component
macromolecular complex
protein complex
proton-transporting two-sector atpase complex, proton-transporting domain
Function
transmembrane transporter activity
substrate-specific transmembrane transporter activity
ion transmembrane transporter activity
cation transmembrane transporter activity
inorganic cation transmembrane transporter activity
monovalent inorganic cation transmembrane transporter activity
hydrogen ion transmembrane transporter activity
transporter activity
Process
purine nucleotide metabolic process
purine nucleotide biosynthetic process
purine nucleoside triphosphate biosynthetic process
purine ribonucleoside triphosphate biosynthetic process
metabolic process
nitrogen compound metabolic process
cellular nitrogen compound metabolic process
nucleobase, nucleoside, nucleotide and nucleic acid metabolic process
nucleobase, nucleoside and nucleotide metabolic process
nucleoside phosphate metabolic process
nucleotide metabolic process
atp synthesis coupled proton transport
atp biosynthetic process
Cellular Location
  1. Cell membrane
  2. Endosome membrane
  3. Multi-pass membrane protein
Gene Properties
Chromosome Location Chromosome:1
Locus 12q24.31
SNPs ATP6V0A2
Gene Sequence
>2571 bp
ATGGGGTCCCTGTTCCGGAGCGAGACCATGTGCCTGGCGCAGCTCTTCCTGCAGTCGGGC
ACGGCCTACGAGTGCCTCAGCGCCCTGGGCGAGAAAGGCCTGGTCCAGTTCCGAGACCTC
AACCAGAACGTAAGTTCTTTCCAAAGAAAATTTGTTGGTGAGGTGAAGAGGTGTGAAGAG
CTAGAGCGAATATTGGTGTATTTGGTACAGGAAATTAATAGAGCTGATATTCCCCTTCCT
GAAGGAGAGGCCAGCCCTCCTGCGCCACCCCTGAAACAGGTTCTAGAAATGCAGGAGCAG
TTGCAGAAGCTCGAGGTTGAACTGAGAGAAGTCACTAAGAACAAGGAGAAACTGAGGAAA
AACTTGCTGGAACTGATAGAGTACACTCACATGCTGAGAGTGACAAAGACCTTTGTGAAA
CGCAACGTTGAGTTTGAACCCACTTATGAAGAATTCCCTTCCTTAGAGAGCGATTCTTTG
TTGGATTACAGCTGTATGCAGAGGCTGGGAGCAAAACTGGGATTTGTGTCTGGCCTAATT
AACCAAGGAAAAGTGGAAGCATTTGAAAAAATGTTGTGGAGAGTCTGCAAAGGGTACACC
ATCGTGTCCTATGCAGAACTGGATGAATCCCTTGAAGACCCTGAAACAGGGGAAGTCATA
AAATGGTATGTCTTTTTAATATCCTTTTGGGGAGAGCAGATTGGCCACAAGGTTAAGAAG
ATATGTGATTGCTACCACTGCCACGTGTACCCCTATCCAAACACAGCCGAGGAGCGGAGG
GAGATCCAGGAGGGGCTGAACACCCGCATCCAGGATCTCTACACTGTACTGCACAAAACC
GAGGACTATTTGAGGCAAGTGCTATGTAAAGCCGCCGAGTCTGTCTACAGCCGTGTGATC
CAGGTGAAGAAAATGAAGGCCATCTATCACATGCTGAACATGTGCAGCTTTGACGTGACC
AACAAGTGCCTCATTGCTGAAGTCTGGTGTCCCGAGGCGGATCTGCAGGACCTGCGCCGG
GCACTGGAGGAGGGCTCGAGAGAGAGTGGTGCTACAATCCCCTCATTCATGAATATAATC
CCCACAAAAGAAACACCCCCCACTCGGATCCGCACCAACAAATTCACCGAGGGATTTCAG
AACATCGTGGATGCTTATGGAGTCGGAAGCTACAGAGAAGTCAATCCAGCTCTCTTTACC
ATCATCACCTTCCCGTTTTTATTTGCTGTGATGTTTGGAGACTTCGGACATGGCTTTGTG
ATGTTTTTATTTGCCCTCTTGTGGGTGTTAAATGAAAATCATCCCAGACTAAATCAGTCA
CAAGAGATCATGAGGATGTTTTTTAATGGCCGGTACATCCTCCTGCTGATGGGGCTGTTC
TCAGTGTACACTGGCCTCATCTACAACGACTGCTTTTCAAAGTCAGTCAACCTGTTCGGC
TCTGGGTGGAACGTGTCGGCCATGTACAGCTCCAGCCACCCACCCGCAGAGCATAAGAAG
ATGGTGCTTTGGAACGACAGCGTCGTTAGACACAACAGCATTTTGCAGCTGGATCCAAGC
ATTCCTGGAGTGTTCCGAGGCCCTTATCCCCTTGGCATTGATCCTATTTGGAACTTGGCC
ACAAATCGCCTCACTTTTCTAAACTCTTTCAAAATGAAAATGTCCGTGATTTTAGGAATC
ATTCATATGACTTTTGGAGTCATTCTGGGAATATTTAACCACTTGCACTTCAGGAAGAAG
TTCAACATTTACCTGGTTTCCATCCCGGAACTTCTCTTCATGCTCTGTATCTTTGGATAC
CTTATATTTATGATTTTCTACAAGTGGCTGGTTTTTTCAGCAGAAACCTCCAGAGTTGCT
CCCAGCATTCTGATTGAATTTATTAACATGTTTTTATTCCCAGCCAGTAAAACAAGTGGC
CTTTACACAGGGCAGGAGTATGTCCAGAGAGTGCTGCTGGTTGTCACAGCATTGTCTGTC
CCTGTCCTCTTCTTGGGAAAGCCACTGTTTTTGTTGTGGCTTCACAATGGGCGTAGTTGC
TTCGGGGTGAACCGGAGTGGCTACACACTTATAAGGAAAGATAGTGAGGAAGAAGTTTCA
TTGCTGGGAAGCCAAGATATAGAAGAGGGAAATCACCAGGTGGAAGATGGATGTAGAGAA
ATGGCGTGTGAAGAGTTTAATTTTGGAGAAATATTAATGACCCAAGTAATCCATTCCATC
GAGTACTGTCTGGGATGCATCTCCAACACCGCCTCCTACCTGAGGCTCTGGGCGCTTAGC
CTGGCTCACGCACAGTTGTCTGATGTCCTGTGGGCCATGCTGATGCGCGTGGGCCTCCGC
GTTGACACCACCTATGGCGTCTTGCTACTGCTCCCGGTTATCGCGCTCTTTGCAGTTTTG
ACCATTTTCATCCTTCTGATCATGGAAGGGCTTTCTGCGTTTCTTCACGCCATACGCCTC
CACTGGGTAGAATTTCAGAACAAATTCTACGTTGGTGCAGGCACCAAATTTGTTCCTTTC
TCATTCAGTCTACTTTCATCAAAGTTCAATAACGACGACAGTGTGGCATGA
Protein Properties
Number of Residues 856
Molecular Weight 98081.5
Theoretical pI 6.59
Pfam Domain Function
Signals
  • None
Transmembrane Regions
  • 406-426
  • 452-472
  • 555-575
  • 592-612
  • 623-643
  • 651-671
  • 754-774
  • 787-807
Protein Sequence
>V-type proton ATPase 116 kDa subunit a isoform 2
MGSLFRSETMCLAQLFLQSGTAYECLSALGEKGLVQFRDLNQNVSSFQRKFVGEVKRCEE
LERILVYLVQEINRADIPLPEGEASPPAPPLKQVLEMQEQLQKLEVELREVTKNKEKLRK
NLLELIEYTHMLRVTKTFVKRNVEFEPTYEEFPSLESDSLLDYSCMQRLGAKLGFVSGLI
NQGKVEAFEKMLWRVCKGYTIVSYAELDESLEDPETGEVIKWYVFLISFWGEQIGHKVKK
ICDCYHCHVYPYPNTAEERREIQEGLNTRIQDLYTVLHKTEDYLRQVLCKAAESVYSRVI
QVKKMKAIYHMLNMCSFDVTNKCLIAEVWCPEADLQDLRRALEEGSRESGATIPSFMNII
PTKETPPTRIRTNKFTEGFQNIVDAYGVGSYREVNPALFTIITFPFLFAVMFGDFGHGFV
MFLFALLLVLNENHPRLNQSQEIMRMFFNGRYILLLMGLFSVYTGLIYNDCFSKSVNLFG
SGWNVSAMYSSSHPPAEHKKMVLWNDSVVRHNSILQLDPSIPGVFRGPYPLGIDPIWNLA
TNRLTFLNSFKMKMSVILGIIHMTFGVILGIFNHLHFRKKFNIYLVSIPELLFMLCIFGY
LIFMIFYKWLVFSAETSRVAPSILIEFINMFLFPASKTSGLYTGQEYVQRVLLVVTALSV
PVLFLGKPLFLLWLHNGRSCFGVNRSGYTLIRKDSEEEVSLLGSQDIEEGNHQVEDGCRE
MACEEFNFGEILMTQVIHSIEYCLGCISNTASYLRLWALSLAHAQLSDVLWAMLMRVGLR
VDTTYGVLLLLPVIALFAVLTIFILLIMEGLSAFLHAIRLHWVEFQNKFYVGAGTKFVPF
SFSLLSSKFNNDDSVA
GenBank ID Protein 4151944
UniProtKB/Swiss-Prot ID Q9Y487
UniProtKB/Swiss-Prot Entry Name VPP2_HUMAN
PDB IDs Not Available
GenBank Gene ID AF112972
GeneCard ID ATP6V0A2
GenAtlas ID Not Available
HGNC ID Not Available
References
General References
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  3. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861 ]
  4. Dephoure N, Zhou C, Villen J, Beausoleil SA, Bakalarski CE, Elledge SJ, Gygi SP: A quantitative atlas of mitotic phosphorylation. Proc Natl Acad Sci U S A. 2008 Aug 5;105(31):10762-7. doi: 10.1073/pnas.0805139105. Epub 2008 Jul 31. [PubMed:18669648 ]
  5. Olsen JV, Blagoev B, Gnad F, Macek B, Kumar C, Mortensen P, Mann M: Global, in vivo, and site-specific phosphorylation dynamics in signaling networks. Cell. 2006 Nov 3;127(3):635-48. [PubMed:17081983 ]
  6. Matsuoka S, Ballif BA, Smogorzewska A, McDonald ER 3rd, Hurov KE, Luo J, Bakalarski CE, Zhao Z, Solimini N, Lerenthal Y, Shiloh Y, Gygi SP, Elledge SJ: ATM and ATR substrate analysis reveals extensive protein networks responsive to DNA damage. Science. 2007 May 25;316(5828):1160-6. [PubMed:17525332 ]
  7. Hurtado-Lorenzo A, Skinner M, El Annan J, Futai M, Sun-Wada GH, Bourgoin S, Casanova J, Wildeman A, Bechoua S, Ausiello DA, Brown D, Marshansky V: V-ATPase interacts with ARNO and Arf6 in early endosomes and regulates the protein degradative pathway. Nat Cell Biol. 2006 Feb;8(2):124-36. Epub 2006 Jan 15. [PubMed:16415858 ]
  8. Ntrivalas E, Gilman-Sachs A, Kwak-Kim J, Beaman K: The N-terminus domain of the a2 isoform of vacuolar ATPase can regulate interleukin-1beta production from mononuclear cells in co-culture with JEG-3 choriocarcinoma cells. Am J Reprod Immunol. 2007 Mar;57(3):201-9. [PubMed:17295899 ]
  9. Kornak U, Reynders E, Dimopoulou A, van Reeuwijk J, Fischer B, Rajab A, Budde B, Nurnberg P, Foulquier F, Lefeber D, Urban Z, Gruenewald S, Annaert W, Brunner HG, van Bokhoven H, Wevers R, Morava E, Matthijs G, Van Maldergem L, Mundlos S: Impaired glycosylation and cutis laxa caused by mutations in the vesicular H+-ATPase subunit ATP6V0A2. Nat Genet. 2008 Jan;40(1):32-4. Epub 2007 Dec 23. [PubMed:18157129 ]