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Q64446 (ATP7B_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 135. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (2) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Copper-transporting ATPase 2

EC=3.6.3.54
Alternative name(s):
Copper pump 2
Wilson disease-associated protein homolog
Gene names
Name:Atp7b
Synonyms:Wnd
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length1462 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile By similarity.

Catalytic activity

ATP + H2O + Cu+(Side 1) = ADP + phosphate + Cu+(Side 2).

Subunit structure

Monomer. Interacts with COMMD1/MURR1 By similarity. Interacts with DCTN4, in a copper-dependent manner. Interacts with ATOX1 By similarity.

Subcellular location

Golgi apparatustrans-Golgi network membrane; Multi-pass membrane protein By similarity. Note: Predominantly found in the trans-Golgi network (TGN). Not redistributed to the plasma membrane in response to elevated copper levels By similarity.

Tissue specificity

Detected in liver and kidney.

Domain

Each HMA domain can bind a copper ion, they are tightly packed and closely interact with each other. Wild-type ATP7B can usually be loaded with an average 5.5 copper atoms per molecule By similarity.

Involvement in disease

Defects in Atp7b are the cause of the toxic milk mouse mutant (tx) phenotype, characterized by accumulation of copper in the liver in a manner similar to that observed in patients with Wilson disease.

Sequence similarities

Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily. [View classification]

Contains 6 HMA domains.

Ontologies

Keywords
   Biological processCopper transport
Ion transport
Transport
   Cellular componentGolgi apparatus
Membrane
   DiseaseDisease mutation
   DomainRepeat
Transmembrane
Transmembrane helix
   LigandATP-binding
Copper
Magnesium
Metal-binding
Nucleotide-binding
   Molecular functionHydrolase
   Technical termComplete proteome
Reference proteome
Gene Ontology (GO)
   Biological_processcellular copper ion homeostasis

Inferred from mutant phenotype PubMed 10441329PubMed 11085952PubMed 1588441PubMed 2845190PubMed 6863890PubMed 8040371PubMed 8257436PubMed 9392450PubMed 9484715. Source: MGI

cellular zinc ion homeostasis

Inferred from mutant phenotype PubMed 8040371PubMed 8257436PubMed 9392450PubMed 9484715. Source: MGI

copper ion export

Inferred from direct assay PubMed 15634671. Source: GOC

copper ion import

Inferred from electronic annotation. Source: Ensembl

copper ion transport

Inferred from direct assay PubMed 11237756PubMed 15634671. Source: MGI

intracellular copper ion transport

Inferred from mutant phenotype PubMed 16436657. Source: MGI

lactation

Inferred from mutant phenotype PubMed 1588441PubMed 6863890. Source: MGI

response to copper ion

Inferred from electronic annotation. Source: Ensembl

sequestering of calcium ion

Inferred from electronic annotation. Source: Ensembl

   Cellular_componentGolgi apparatus

Inferred from electronic annotation. Source: UniProtKB-SubCell

basolateral plasma membrane

Inferred from electronic annotation. Source: Ensembl

cytoplasmic membrane-bounded vesicle

Inferred from electronic annotation. Source: Ensembl

integral component of membrane

Inferred from electronic annotation. Source: UniProtKB-KW

late endosome

Inferred from sequence or structural similarity. Source: UniProtKB

membrane

Inferred from direct assay PubMed 10441329PubMed 15634671. Source: MGI

perinuclear region of cytoplasm

Inferred from electronic annotation. Source: Ensembl

trans-Golgi network

Inferred from direct assay PubMed 11085952. Source: MGI

   Molecular_functionATP binding

Inferred from sequence or structural similarity. Source: HGNC

copper ion binding

Inferred from sequence or structural similarity. Source: HGNC

copper-exporting ATPase activity

Inferred from direct assay PubMed 15634671. Source: MGI

copper-transporting ATPase activity

Inferred from direct assay PubMed 11237756. Source: MGI

Complete GO annotation...

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 14621462Copper-transporting ATPase 2
PRO_0000046315

Regions

Topological domain1 – 655655Cytoplasmic Potential
Transmembrane656 – 67722Helical; Potential
Topological domain678 – 69922Extracellular Potential
Transmembrane700 – 71920Helical; Potential
Topological domain720 – 7267Cytoplasmic Potential
Transmembrane727 – 74721Helical; Potential
Topological domain748 – 76619Extracellular Potential
Transmembrane767 – 78721Helical; Potential
Topological domain788 – 921134Cytoplasmic Potential
Transmembrane922 – 94423Helical; Potential
Topological domain945 – 97430Extracellular Potential
Transmembrane975 – 99622Helical; Potential
Topological domain997 – 1319323Cytoplasmic Potential
Transmembrane1320 – 133718Helical; Potential
Topological domain1338 – 134811Extracellular Potential
Transmembrane1349 – 136820Helical; Potential
Topological domain1369 – 146294Cytoplasmic Potential
Domain69 – 13567HMA 1
Domain154 – 22067HMA 2
Domain268 – 33467HMA 3
Domain362 – 42867HMA 4
Domain491 – 55767HMA 5
Domain567 – 63367HMA 6

Sites

Active site102914-aspartylphosphate intermediate By similarity
Metal binding791Copper 1 By similarity
Metal binding821Copper 1 By similarity
Metal binding1641Copper 2 By similarity
Metal binding1671Copper 2 By similarity
Metal binding2781Copper 3 By similarity
Metal binding2811Copper 3 By similarity
Metal binding3721Copper 4 By similarity
Metal binding5011Copper 5 By similarity
Metal binding5041Copper 5 By similarity
Metal binding5771Copper 6 By similarity
Metal binding5801Copper 6 By similarity
Metal binding12641Magnesium By similarity
Metal binding12681Magnesium By similarity

Natural variations

Natural variant13561M → V in tx mice.

Experimental info

Sequence conflict107 – 1082SA → KH in AAC52852. Ref.1

Sequences

Sequence LengthMass (Da)Tools
Q64446 [UniParc].

Last modified July 27, 2011. Version 2.
Checksum: BEC219F80EB74214

FASTA1,462157,190
        10         20         30         40         50         60 
MDPRKNLASV GTMPEQERQV TAKEASRKIL SKLALPGRPW EQSMKQSFAF DNVGYEGGLD 

        70         80         90        100        110        120 
STSSSPAATD VVNILGMTCH SCVKSIEDRI SSLKGIVNIK VSLEQGSATV RYVPSVMNLQ 

       130        140        150        160        170        180 
QICLQIEDMG FEASAAEGKA ASWPSRSSPA QEAVVKLRVE GMTCQSCVSS IEGKIRKLQG 

       190        200        210        220        230        240 
VVRIKVSLSN QEAVITYQPY LIQPEDLRDH ICDMGFEAAI KNRTAPLRLG PIDVNKLEST 

       250        260        270        280        290        300 
NLKKETVSPV QISNHFETLG HQGSYLATLP LRIDGMHCKS CVLNIEGNIG QLPGVQNIHV 

       310        320        330        340        350        360 
SLENKTAQIQ YDPSCVTPMF LQTAIEALPP GHFKVSLPDG VEENEPQSGS SQRHQEQGPG 

       370        380        390        400        410        420 
RTAVLTISGI TCASSVQPIE DMLSQRKGVQ QTSISLAEGT GAVLYDPSIV SLDELRTAVE 

       430        440        450        460        470        480 
DMGFEVSVNS ETFTINPVRN FKSGNSVPQT MGDIAGSVQK MAPDTRGLPT HQGPGHSSET 

       490        500        510        520        530        540 
PSSPGATASQ KCFVQIKGMT CASCVSNIER SLQRHAGILS VLVALMSGKA EVKYDPEIIQ 

       550        560        570        580        590        600 
SPRIAQLIQD LGFEASVMED NTVSEGDIEL IITGMTCASC VHNIESKLTR TNGITYASVA 

       610        620        630        640        650        660 
LATSKAHVKF DPEIVGPRDI IKIIEEIGFH ASLAQRNPNA HHLDHKTEIK QWKKSFLCSL 

       670        680        690        700        710        720 
VFGIPVMGLM VYMLIPSSTP QETMVLDHNI IPGLSVLNLI FFILCTFVQF LGGWYFYVQA 

       730        740        750        760        770        780 
YKSLRHRSAN MDVLIVLATT IAYAYSLVIL VVAVAEKAEK SPVTFFDTPP MLFVFIALGR 

       790        800        810        820        830        840 
WLEHVAKSKT SEALAKLMSL QATEATVVTL GEDNLILREE QVPMELVQRG DVIKVVPGGK 

       850        860        870        880        890        900 
FPVDGKVLEG NTMADESLIT GEAMPVTKKP GSIVIAGSIN AHGSVLLKAT HVGNDTTLAQ 

       910        920        930        940        950        960 
IVKLVEEAQM SKAPIQQLAD RFSGYFVPFI IIISTLTLVV WIVIGFVDFG VVQKYFPSPS 

       970        980        990       1000       1010       1020 
KHISQTEVII RFAFQTSITV LCIACPCSLG LATPTAVMVG TGVAAQNGVL IKGGKPLEMA 

      1030       1040       1050       1060       1070       1080 
HKIKTVMFDK TGTITHGVPR VMRFLLLADV ATLPLRKVLA VVGTAEASSE HPLGVAVTKY 

      1090       1100       1110       1120       1130       1140 
CKEELGTETL GYSTDFQAVP GCGISCKVSN VEGILARSDL TAHPVGVGNP PTGEGAGPQT 

      1150       1160       1170       1180       1190       1200 
FSVLIGNREW MRRNGLTISS DISDAMTDHE MKGQTAILVA IDGVLCGMIA IADAVKPEAA 

      1210       1220       1230       1240       1250       1260 
LAIYTLKSMG VDVALITGDN RKTARAIATQ VGINKVFAEV LPSHKVAKVQ ELQNEGKKVA 

      1270       1280       1290       1300       1310       1320 
MVGDGVNDSP ALAQADVGIA IGTGTDVAIE AADVVLIRND LLDVVASIHL SKRTVRRIRV 

      1330       1340       1350       1360       1370       1380 
NLVLALIYNM VGIPIAAGVF MPIGIVLQPW MGSAAMAASS VSVVLSSLQL KCYRKPDLER 

      1390       1400       1410       1420       1430       1440 
YEAQAHGRMK PLSASQVSVH IGMDDRRRDS PRATAWDQVS YVSQVSLSSL TSDRLSRHGG 

      1450       1460 
AAEDGGDKWS LLLSDRDEEQ CI 

« Hide

References

« Hide 'large scale' references
[1]"The toxic milk mouse is a murine model of Wilson disease."
Theophilos M.B., Cox D.W., Mercer J.F.B.
Hum. Mol. Genet. 5:1619-1624(1996) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Strain: DAT.
Tissue: Liver.
[2]"Lineage-specific biology revealed by a finished genome assembly of the mouse."
Church D.M., Goodstadt L., Hillier L.W., Zody M.C., Goldstein S., She X., Bult C.J., Agarwala R., Cherry J.L., DiCuccio M., Hlavina W., Kapustin Y., Meric P., Maglott D., Birtle Z., Marques A.C., Graves T., Zhou S. expand/collapse author list , Teague B., Potamousis K., Churas C., Place M., Herschleb J., Runnheim R., Forrest D., Amos-Landgraf J., Schwartz D.C., Cheng Z., Lindblad-Toh K., Eichler E.E., Ponting C.P.
PLoS Biol. 7:E1000112-E1000112(2009) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
Strain: C57BL/6J.
[3]Mural R.J., Adams M.D., Myers E.W., Smith H.O., Venter J.C.
Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
U38477 mRNA. Translation: AAC52852.1.
AL590619, AC163439 Genomic DNA. Translation: CAM22052.1.
CH466580 Genomic DNA. Translation: EDL32910.1.
RefSeqNP_031537.2. NM_007511.2.
UniGeneMm.87854.

3D structure databases

ProteinModelPortalQ64446.
SMRQ64446. Positions 68-1378.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid198269. 1 interaction.
IntActQ64446. 1 interaction.
MINTMINT-4088816.
STRING10090.ENSMUSP00000006742.

PTM databases

PhosphoSiteQ64446.

Proteomic databases

PaxDbQ64446.
PRIDEQ64446.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000006742; ENSMUSP00000006742; ENSMUSG00000006567.
GeneID11979.
KEGGmmu:11979.
UCSCuc009lck.1. mouse.

Organism-specific databases

CTD540.
MGIMGI:103297. Atp7b.

Phylogenomic databases

eggNOGCOG2217.
GeneTreeENSGT00530000063773.
HOGENOMHOG000250397.
HOVERGENHBG050616.
InParanoidB1AQ56.
KOK17686.
OMAMRRNGLT.
OrthoDBEOG7C2R0G.
TreeFamTF300460.

Enzyme and pathway databases

BRENDA3.6.3.4. 2681.

Gene expression databases

BgeeQ64446.
CleanExMM_ATP7B.
GenevestigatorQ64446.

Family and domain databases

Gene3D2.70.150.10. 1 hit.
3.40.1110.10. 1 hit.
3.40.50.1000. 2 hits.
InterProIPR023299. ATPase_P-typ_cyto_domN.
IPR018303. ATPase_P-typ_P_site.
IPR008250. ATPase_P-typ_transduc_dom_A.
IPR027256. Cation_transp_P-typ_ATPase_IB.
IPR001757. Cation_transp_P_typ_ATPase.
IPR023214. HAD-like_dom.
IPR017969. Heavy-metal-associated_CS.
IPR006121. HeavyMe-assoc_HMA.
IPR006122. HMA_Cu_ion-bd.
[Graphical view]
PfamPF00122. E1-E2_ATPase. 1 hit.
PF00403. HMA. 6 hits.
PF00702. Hydrolase. 1 hit.
[Graphical view]
PRINTSPR00119. CATATPASE.
SUPFAMSSF55008. SSF55008. 6 hits.
SSF56784. SSF56784. 2 hits.
TIGRFAMsTIGR01525. ATPase-IB_hvy. 1 hit.
TIGR01494. ATPase_P-type. 2 hits.
TIGR00003. TIGR00003. 5 hits.
PROSITEPS00154. ATPASE_E1_E2. 1 hit.
PS01047. HMA_1. 5 hits.
PS50846. HMA_2. 6 hits.
[Graphical view]
ProtoNetSearch...

Other

NextBio280115.
PROQ64446.
SOURCESearch...

Entry information

Entry nameATP7B_MOUSE
AccessionPrimary (citable) accession number: Q64446
Secondary accession number(s): B1AQ56
Entry history
Integrated into UniProtKB/Swiss-Prot: December 8, 2000
Last sequence update: July 27, 2011
Last modified: April 16, 2014
This is version 135 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot