SubmitCancel

Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.

Q64446

- ATP7B_MOUSE

UniProt

Q64446 - ATP7B_MOUSE

(max 400 entries)x

Your basket is currently empty.

Select item(s) and click on "Add to basket" to create your own collection here
(400 entries max)

Protein
Copper-transporting ATPase 2
Gene
Atp7b, Wnd
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

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

Catalytic activityi

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

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi79 – 791Copper 1 By similarity
Metal bindingi82 – 821Copper 1 By similarity
Metal bindingi164 – 1641Copper 2 By similarity
Metal bindingi167 – 1671Copper 2 By similarity
Metal bindingi278 – 2781Copper 3 By similarity
Metal bindingi281 – 2811Copper 3 By similarity
Metal bindingi372 – 3721Copper 4 By similarity
Metal bindingi501 – 5011Copper 5 By similarity
Metal bindingi504 – 5041Copper 5 By similarity
Metal bindingi577 – 5771Copper 6 By similarity
Metal bindingi580 – 5801Copper 6 By similarity
Active sitei1029 – 102914-aspartylphosphate intermediate By similarity
Metal bindingi1264 – 12641Magnesium By similarity
Metal bindingi1268 – 12681Magnesium By similarity

GO - Molecular functioni

  1. ATP binding Source: HGNC
  2. copper ion binding Source: HGNC
  3. copper-exporting ATPase activity Source: MGI
  4. copper-transporting ATPase activity Source: MGI

GO - Biological processi

  1. cellular copper ion homeostasis Source: MGI
  2. cellular zinc ion homeostasis Source: MGI
  3. copper ion export Source: GOC
  4. copper ion import Source: Ensembl
  5. copper ion transport Source: MGI
  6. intracellular copper ion transport Source: MGI
  7. lactation Source: MGI
  8. response to copper ion Source: Ensembl
  9. sequestering of calcium ion Source: Ensembl
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase

Keywords - Biological processi

Copper transport, Ion transport, Transport

Keywords - Ligandi

ATP-binding, Copper, Magnesium, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

BRENDAi3.6.3.4. 2681.

Names & Taxonomyi

Protein namesi
Recommended name:
Copper-transporting ATPase 2 (EC:3.6.3.54)
Alternative name(s):
Copper pump 2
Wilson disease-associated protein homolog
Gene namesi
Name:Atp7b
Synonyms:Wnd
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 8

Organism-specific databases

MGIiMGI:103297. Atp7b.

Subcellular locationi

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.

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 655655Cytoplasmic Reviewed prediction
Add
BLAST
Transmembranei656 – 67722Helical; Reviewed prediction
Add
BLAST
Topological domaini678 – 69922Extracellular Reviewed prediction
Add
BLAST
Transmembranei700 – 71920Helical; Reviewed prediction
Add
BLAST
Topological domaini720 – 7267Cytoplasmic Reviewed prediction
Transmembranei727 – 74721Helical; Reviewed prediction
Add
BLAST
Topological domaini748 – 76619Extracellular Reviewed prediction
Add
BLAST
Transmembranei767 – 78721Helical; Reviewed prediction
Add
BLAST
Topological domaini788 – 921134Cytoplasmic Reviewed prediction
Add
BLAST
Transmembranei922 – 94423Helical; Reviewed prediction
Add
BLAST
Topological domaini945 – 97430Extracellular Reviewed prediction
Add
BLAST
Transmembranei975 – 99622Helical; Reviewed prediction
Add
BLAST
Topological domaini997 – 1319323Cytoplasmic Reviewed prediction
Add
BLAST
Transmembranei1320 – 133718Helical; Reviewed prediction
Add
BLAST
Topological domaini1338 – 134811Extracellular Reviewed prediction
Add
BLAST
Transmembranei1349 – 136820Helical; Reviewed prediction
Add
BLAST
Topological domaini1369 – 146294Cytoplasmic Reviewed prediction
Add
BLAST

GO - Cellular componenti

  1. basolateral plasma membrane Source: Ensembl
  2. cytoplasmic membrane-bounded vesicle Source: Ensembl
  3. integral component of membrane Source: UniProtKB-KW
  4. late endosome Source: UniProtKB
  5. membrane Source: MGI
  6. perinuclear region of cytoplasm Source: Ensembl
  7. trans-Golgi network Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

Pathology & Biotechi

Involvement in diseasei

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.

Keywords - Diseasei

Disease mutation

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 14621462Copper-transporting ATPase 2
PRO_0000046315Add
BLAST

Proteomic databases

MaxQBiQ64446.
PaxDbiQ64446.
PRIDEiQ64446.

PTM databases

PhosphoSiteiQ64446.

Expressioni

Tissue specificityi

Detected in liver and kidney.

Gene expression databases

BgeeiQ64446.
CleanExiMM_ATP7B.
GenevestigatoriQ64446.

Interactioni

Subunit structurei

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

Protein-protein interaction databases

BioGridi198269. 1 interaction.
IntActiQ64446. 1 interaction.
MINTiMINT-4088816.
STRINGi10090.ENSMUSP00000006742.

Structurei

3D structure databases

ProteinModelPortaliQ64446.
SMRiQ64446. Positions 68-635, 647-1376.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini69 – 13567HMA 1
Add
BLAST
Domaini154 – 22067HMA 2
Add
BLAST
Domaini268 – 33467HMA 3
Add
BLAST
Domaini362 – 42867HMA 4
Add
BLAST
Domaini491 – 55767HMA 5
Add
BLAST
Domaini567 – 63367HMA 6
Add
BLAST

Domaini

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.

Sequence similaritiesi

Contains 6 HMA domains.

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiCOG2217.
GeneTreeiENSGT00530000063773.
HOGENOMiHOG000250397.
HOVERGENiHBG050616.
InParanoidiB1AQ56.
KOiK17686.
OMAiVAPQKCF.
OrthoDBiEOG7C2R0G.
TreeFamiTF300460.

Family and domain databases

Gene3Di2.70.150.10. 1 hit.
3.40.1110.10. 1 hit.
3.40.50.1000. 2 hits.
InterProiIPR023299. 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]
PfamiPF00122. E1-E2_ATPase. 1 hit.
PF00403. HMA. 6 hits.
PF00702. Hydrolase. 1 hit.
[Graphical view]
PRINTSiPR00119. CATATPASE.
SUPFAMiSSF55008. SSF55008. 6 hits.
SSF56784. SSF56784. 2 hits.
TIGRFAMsiTIGR01525. ATPase-IB_hvy. 1 hit.
TIGR01494. ATPase_P-type. 2 hits.
TIGR00003. TIGR00003. 5 hits.
PROSITEiPS00154. ATPASE_E1_E2. 1 hit.
PS01047. HMA_1. 5 hits.
PS50846. HMA_2. 6 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Q64446-1 [UniParc]FASTAAdd to Basket

« Hide

MDPRKNLASV GTMPEQERQV TAKEASRKIL SKLALPGRPW EQSMKQSFAF     50
DNVGYEGGLD STSSSPAATD VVNILGMTCH SCVKSIEDRI SSLKGIVNIK 100
VSLEQGSATV RYVPSVMNLQ QICLQIEDMG FEASAAEGKA ASWPSRSSPA 150
QEAVVKLRVE GMTCQSCVSS IEGKIRKLQG VVRIKVSLSN QEAVITYQPY 200
LIQPEDLRDH ICDMGFEAAI KNRTAPLRLG PIDVNKLEST NLKKETVSPV 250
QISNHFETLG HQGSYLATLP LRIDGMHCKS CVLNIEGNIG QLPGVQNIHV 300
SLENKTAQIQ YDPSCVTPMF LQTAIEALPP GHFKVSLPDG VEENEPQSGS 350
SQRHQEQGPG RTAVLTISGI TCASSVQPIE DMLSQRKGVQ QTSISLAEGT 400
GAVLYDPSIV SLDELRTAVE DMGFEVSVNS ETFTINPVRN FKSGNSVPQT 450
MGDIAGSVQK MAPDTRGLPT HQGPGHSSET PSSPGATASQ KCFVQIKGMT 500
CASCVSNIER SLQRHAGILS VLVALMSGKA EVKYDPEIIQ SPRIAQLIQD 550
LGFEASVMED NTVSEGDIEL IITGMTCASC VHNIESKLTR TNGITYASVA 600
LATSKAHVKF DPEIVGPRDI IKIIEEIGFH ASLAQRNPNA HHLDHKTEIK 650
QWKKSFLCSL VFGIPVMGLM VYMLIPSSTP QETMVLDHNI IPGLSVLNLI 700
FFILCTFVQF LGGWYFYVQA YKSLRHRSAN MDVLIVLATT IAYAYSLVIL 750
VVAVAEKAEK SPVTFFDTPP MLFVFIALGR WLEHVAKSKT SEALAKLMSL 800
QATEATVVTL GEDNLILREE QVPMELVQRG DVIKVVPGGK FPVDGKVLEG 850
NTMADESLIT GEAMPVTKKP GSIVIAGSIN AHGSVLLKAT HVGNDTTLAQ 900
IVKLVEEAQM SKAPIQQLAD RFSGYFVPFI IIISTLTLVV WIVIGFVDFG 950
VVQKYFPSPS KHISQTEVII RFAFQTSITV LCIACPCSLG LATPTAVMVG 1000
TGVAAQNGVL IKGGKPLEMA HKIKTVMFDK TGTITHGVPR VMRFLLLADV 1050
ATLPLRKVLA VVGTAEASSE HPLGVAVTKY CKEELGTETL GYSTDFQAVP 1100
GCGISCKVSN VEGILARSDL TAHPVGVGNP PTGEGAGPQT FSVLIGNREW 1150
MRRNGLTISS DISDAMTDHE MKGQTAILVA IDGVLCGMIA IADAVKPEAA 1200
LAIYTLKSMG VDVALITGDN RKTARAIATQ VGINKVFAEV LPSHKVAKVQ 1250
ELQNEGKKVA MVGDGVNDSP ALAQADVGIA IGTGTDVAIE AADVVLIRND 1300
LLDVVASIHL SKRTVRRIRV NLVLALIYNM VGIPIAAGVF MPIGIVLQPW 1350
MGSAAMAASS VSVVLSSLQL KCYRKPDLER YEAQAHGRMK PLSASQVSVH 1400
IGMDDRRRDS PRATAWDQVS YVSQVSLSSL TSDRLSRHGG AAEDGGDKWS 1450
LLLSDRDEEQ CI 1462
Length:1,462
Mass (Da):157,190
Last modified:July 27, 2011 - v2
Checksum:iBEC219F80EB74214
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti1356 – 13561M → V in tx mice.

Sequence conflict

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti107 – 1082SA → KH in AAC52852. 1 Publication

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
U38477 mRNA. Translation: AAC52852.1.
AL590619, AC163439 Genomic DNA. Translation: CAM22052.1.
CH466580 Genomic DNA. Translation: EDL32910.1.
CCDSiCCDS22168.1.
RefSeqiNP_031537.2. NM_007511.2.
UniGeneiMm.87854.

Genome annotation databases

EnsembliENSMUST00000006742; ENSMUSP00000006742; ENSMUSG00000006567.
GeneIDi11979.
KEGGimmu:11979.
UCSCiuc009lck.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
U38477 mRNA. Translation: AAC52852.1 .
AL590619 , AC163439 Genomic DNA. Translation: CAM22052.1 .
CH466580 Genomic DNA. Translation: EDL32910.1 .
CCDSi CCDS22168.1.
RefSeqi NP_031537.2. NM_007511.2.
UniGenei Mm.87854.

3D structure databases

ProteinModelPortali Q64446.
SMRi Q64446. Positions 68-635, 647-1376.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 198269. 1 interaction.
IntActi Q64446. 1 interaction.
MINTi MINT-4088816.
STRINGi 10090.ENSMUSP00000006742.

PTM databases

PhosphoSitei Q64446.

Proteomic databases

MaxQBi Q64446.
PaxDbi Q64446.
PRIDEi Q64446.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000006742 ; ENSMUSP00000006742 ; ENSMUSG00000006567 .
GeneIDi 11979.
KEGGi mmu:11979.
UCSCi uc009lck.1. mouse.

Organism-specific databases

CTDi 540.
MGIi MGI:103297. Atp7b.

Phylogenomic databases

eggNOGi COG2217.
GeneTreei ENSGT00530000063773.
HOGENOMi HOG000250397.
HOVERGENi HBG050616.
InParanoidi B1AQ56.
KOi K17686.
OMAi VAPQKCF.
OrthoDBi EOG7C2R0G.
TreeFami TF300460.

Enzyme and pathway databases

BRENDAi 3.6.3.4. 2681.

Miscellaneous databases

NextBioi 280115.
PROi Q64446.
SOURCEi Search...

Gene expression databases

Bgeei Q64446.
CleanExi MM_ATP7B.
Genevestigatori Q64446.

Family and domain databases

Gene3Di 2.70.150.10. 1 hit.
3.40.1110.10. 1 hit.
3.40.50.1000. 2 hits.
InterProi IPR023299. 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 ]
Pfami PF00122. E1-E2_ATPase. 1 hit.
PF00403. HMA. 6 hits.
PF00702. Hydrolase. 1 hit.
[Graphical view ]
PRINTSi PR00119. CATATPASE.
SUPFAMi SSF55008. SSF55008. 6 hits.
SSF56784. SSF56784. 2 hits.
TIGRFAMsi TIGR01525. ATPase-IB_hvy. 1 hit.
TIGR01494. ATPase_P-type. 2 hits.
TIGR00003. TIGR00003. 5 hits.
PROSITEi PS00154. ATPASE_E1_E2. 1 hit.
PS01047. HMA_1. 5 hits.
PS50846. HMA_2. 6 hits.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  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. 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].

Entry informationi

Entry nameiATP7B_MOUSE
AccessioniPrimary (citable) accession number: Q64446
Secondary accession number(s): B1AQ56
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 8, 2000
Last sequence update: July 27, 2011
Last modified: July 9, 2014
This is version 138 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi