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Protein

Copper-transporting ATPase 2

Gene

Atp7b

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: 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).By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi79Copper 1PROSITE-ProRule annotation1
Metal bindingi82Copper 1PROSITE-ProRule annotation1
Metal bindingi164Copper 2PROSITE-ProRule annotation1
Metal bindingi167Copper 2PROSITE-ProRule annotation1
Metal bindingi278Copper 3PROSITE-ProRule annotation1
Metal bindingi281Copper 3PROSITE-ProRule annotation1
Metal bindingi372Copper 4PROSITE-ProRule annotation1
Metal bindingi501Copper 5PROSITE-ProRule annotation1
Metal bindingi504Copper 5PROSITE-ProRule annotation1
Metal bindingi577Copper 6PROSITE-ProRule annotation1
Metal bindingi580Copper 6PROSITE-ProRule annotation1
Active sitei10294-aspartylphosphate intermediateBy similarity1
Metal bindingi1264MagnesiumPROSITE-ProRule annotation1
Metal bindingi1268MagnesiumPROSITE-ProRule annotation1

GO - Molecular functioni

  • ATP binding Source: HGNC
  • copper-exporting ATPase activity Source: MGI
  • copper ion binding Source: HGNC
  • copper-transporting ATPase activity Source: MGI

GO - Biological processi

  • cellular copper ion homeostasis Source: MGI
  • cellular zinc ion homeostasis Source: MGI
  • copper ion import Source: MGI
  • copper ion transport Source: MGI
  • intracellular copper ion transport Source: MGI
  • lactation Source: MGI
  • response to copper ion Source: MGI
  • sequestering of calcium ion Source: MGI
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. 3474.
ReactomeiR-MMU-936837. Ion transport by P-type ATPases.

Names & Taxonomyi

Protein namesi
Recommended name:
Copper-transporting ATPase 2 (EC:3.6.3.54By similarity)
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
Proteomesi
  • UP000000589 Componenti: Chromosome 8

Organism-specific databases

MGIiMGI:103297. Atp7b.

Subcellular locationi

  • Golgi apparatustrans-Golgi network membrane By similarity; Multi-pass membrane protein Sequence analysis
  • Late endosome 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)DescriptionActionsGraphical viewLength
Topological domaini1 – 655CytoplasmicSequence analysisAdd BLAST655
Transmembranei656 – 677HelicalSequence analysisAdd BLAST22
Topological domaini678 – 699ExtracellularSequence analysisAdd BLAST22
Transmembranei700 – 719HelicalSequence analysisAdd BLAST20
Topological domaini720 – 726CytoplasmicSequence analysis7
Transmembranei727 – 747HelicalSequence analysisAdd BLAST21
Topological domaini748 – 766ExtracellularSequence analysisAdd BLAST19
Transmembranei767 – 787HelicalSequence analysisAdd BLAST21
Topological domaini788 – 921CytoplasmicSequence analysisAdd BLAST134
Transmembranei922 – 944HelicalSequence analysisAdd BLAST23
Topological domaini945 – 974ExtracellularSequence analysisAdd BLAST30
Transmembranei975 – 996HelicalSequence analysisAdd BLAST22
Topological domaini997 – 1319CytoplasmicSequence analysisAdd BLAST323
Transmembranei1320 – 1337HelicalSequence analysisAdd BLAST18
Topological domaini1338 – 1348ExtracellularSequence analysisAdd BLAST11
Transmembranei1349 – 1368HelicalSequence analysisAdd BLAST20
Topological domaini1369 – 1462CytoplasmicSequence analysisAdd BLAST94

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Endosome, 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)DescriptionActionsGraphical viewLength
ChainiPRO_00000463151 – 1462Copper-transporting ATPase 2Add BLAST1462

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei478PhosphoserineBy similarity1
Modified residuei483PhosphoserineBy similarity1
Modified residuei1395PhosphoserineBy similarity1
Modified residuei1454PhosphoserineBy similarity1

Keywords - PTMi

Phosphoprotein

Proteomic databases

MaxQBiQ64446.
PaxDbiQ64446.
PRIDEiQ64446.

PTM databases

iPTMnetiQ64446.
PhosphoSitePlusiQ64446.

Expressioni

Tissue specificityi

Detected in liver and kidney.

Gene expression databases

BgeeiENSMUSG00000006567.
CleanExiMM_ATP7B.
ExpressionAtlasiQ64446. baseline and differential.
GenevisibleiQ64446. MM.

Interactioni

Subunit structurei

Monomer. Interacts with COMMD1/MURR1 (By similarity). Interacts with DCTN4, in a copper-dependent manner (By similarity). Interacts with ATOX1 (By similarity). Interacts (via C-terminus) with ZBTB16/PLZF (By similarity).By similarity

Protein-protein interaction databases

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

Structurei

3D structure databases

ProteinModelPortaliQ64446.
SMRiQ64446.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini69 – 135HMA 1PROSITE-ProRule annotationAdd BLAST67
Domaini154 – 220HMA 2PROSITE-ProRule annotationAdd BLAST67
Domaini268 – 334HMA 3PROSITE-ProRule annotationAdd BLAST67
Domaini362 – 428HMA 4PROSITE-ProRule annotationAdd BLAST67
Domaini491 – 557HMA 5PROSITE-ProRule annotationAdd BLAST67
Domaini567 – 633HMA 6PROSITE-ProRule annotationAdd BLAST67

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).By similarity

Sequence similaritiesi

Contains 6 HMA domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG0207. Eukaryota.
COG2217. LUCA.
GeneTreeiENSGT00530000063773.
HOGENOMiHOG000250397.
HOVERGENiHBG050616.
InParanoidiQ64446.
KOiK17686.
OMAiAVAPQKC.
OrthoDBiEOG091G022E.
TreeFamiTF300460.

Family and domain databases

CDDicd00371. HMA. 6 hits.
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.
IPR023214. HAD-like_dom.
IPR017969. Heavy-metal-associated_CS.
IPR006122. HMA_Cu_ion-bd.
IPR006121. HMA_dom.
IPR027256. P-typ_ATPase_IB.
IPR001757. P_typ_ATPase.
[Graphical view]
PfamiPF00122. E1-E2_ATPase. 1 hit.
PF00403. HMA. 6 hits.
[Graphical view]
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

        10         20         30         40         50
MDPRKNLASV GTMPEQERQV TAKEASRKIL SKLALPGRPW EQSMKQSFAF
60 70 80 90 100
DNVGYEGGLD STSSSPAATD VVNILGMTCH SCVKSIEDRI SSLKGIVNIK
110 120 130 140 150
VSLEQGSATV RYVPSVMNLQ QICLQIEDMG FEASAAEGKA ASWPSRSSPA
160 170 180 190 200
QEAVVKLRVE GMTCQSCVSS IEGKIRKLQG VVRIKVSLSN QEAVITYQPY
210 220 230 240 250
LIQPEDLRDH ICDMGFEAAI KNRTAPLRLG PIDVNKLEST NLKKETVSPV
260 270 280 290 300
QISNHFETLG HQGSYLATLP LRIDGMHCKS CVLNIEGNIG QLPGVQNIHV
310 320 330 340 350
SLENKTAQIQ YDPSCVTPMF LQTAIEALPP GHFKVSLPDG VEENEPQSGS
360 370 380 390 400
SQRHQEQGPG RTAVLTISGI TCASSVQPIE DMLSQRKGVQ QTSISLAEGT
410 420 430 440 450
GAVLYDPSIV SLDELRTAVE DMGFEVSVNS ETFTINPVRN FKSGNSVPQT
460 470 480 490 500
MGDIAGSVQK MAPDTRGLPT HQGPGHSSET PSSPGATASQ KCFVQIKGMT
510 520 530 540 550
CASCVSNIER SLQRHAGILS VLVALMSGKA EVKYDPEIIQ SPRIAQLIQD
560 570 580 590 600
LGFEASVMED NTVSEGDIEL IITGMTCASC VHNIESKLTR TNGITYASVA
610 620 630 640 650
LATSKAHVKF DPEIVGPRDI IKIIEEIGFH ASLAQRNPNA HHLDHKTEIK
660 670 680 690 700
QWKKSFLCSL VFGIPVMGLM VYMLIPSSTP QETMVLDHNI IPGLSVLNLI
710 720 730 740 750
FFILCTFVQF LGGWYFYVQA YKSLRHRSAN MDVLIVLATT IAYAYSLVIL
760 770 780 790 800
VVAVAEKAEK SPVTFFDTPP MLFVFIALGR WLEHVAKSKT SEALAKLMSL
810 820 830 840 850
QATEATVVTL GEDNLILREE QVPMELVQRG DVIKVVPGGK FPVDGKVLEG
860 870 880 890 900
NTMADESLIT GEAMPVTKKP GSIVIAGSIN AHGSVLLKAT HVGNDTTLAQ
910 920 930 940 950
IVKLVEEAQM SKAPIQQLAD RFSGYFVPFI IIISTLTLVV WIVIGFVDFG
960 970 980 990 1000
VVQKYFPSPS KHISQTEVII RFAFQTSITV LCIACPCSLG LATPTAVMVG
1010 1020 1030 1040 1050
TGVAAQNGVL IKGGKPLEMA HKIKTVMFDK TGTITHGVPR VMRFLLLADV
1060 1070 1080 1090 1100
ATLPLRKVLA VVGTAEASSE HPLGVAVTKY CKEELGTETL GYSTDFQAVP
1110 1120 1130 1140 1150
GCGISCKVSN VEGILARSDL TAHPVGVGNP PTGEGAGPQT FSVLIGNREW
1160 1170 1180 1190 1200
MRRNGLTISS DISDAMTDHE MKGQTAILVA IDGVLCGMIA IADAVKPEAA
1210 1220 1230 1240 1250
LAIYTLKSMG VDVALITGDN RKTARAIATQ VGINKVFAEV LPSHKVAKVQ
1260 1270 1280 1290 1300
ELQNEGKKVA MVGDGVNDSP ALAQADVGIA IGTGTDVAIE AADVVLIRND
1310 1320 1330 1340 1350
LLDVVASIHL SKRTVRRIRV NLVLALIYNM VGIPIAAGVF MPIGIVLQPW
1360 1370 1380 1390 1400
MGSAAMAASS VSVVLSSLQL KCYRKPDLER YEAQAHGRMK PLSASQVSVH
1410 1420 1430 1440 1450
IGMDDRRRDS PRATAWDQVS YVSQVSLSSL TSDRLSRHGG AAEDGGDKWS
1460
LLLSDRDEEQ CI
Length:1,462
Mass (Da):157,190
Last modified:July 27, 2011 - v2
Checksum:iBEC219F80EB74214
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti107 – 108SA → KH in AAC52852 (PubMed:8894697).Curated2

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural varianti1356M → V in tx mice. 1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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:
EMBLi
GenBanki
DDBJi
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.

3D structure databases

ProteinModelPortaliQ64446.
SMRiQ64446.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

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

PTM databases

iPTMnetiQ64446.
PhosphoSitePlusiQ64446.

Proteomic databases

MaxQBiQ64446.
PaxDbiQ64446.
PRIDEiQ64446.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi540.
MGIiMGI:103297. Atp7b.

Phylogenomic databases

eggNOGiKOG0207. Eukaryota.
COG2217. LUCA.
GeneTreeiENSGT00530000063773.
HOGENOMiHOG000250397.
HOVERGENiHBG050616.
InParanoidiQ64446.
KOiK17686.
OMAiAVAPQKC.
OrthoDBiEOG091G022E.
TreeFamiTF300460.

Enzyme and pathway databases

BRENDAi3.6.3.4. 3474.
ReactomeiR-MMU-936837. Ion transport by P-type ATPases.

Miscellaneous databases

PROiQ64446.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000006567.
CleanExiMM_ATP7B.
ExpressionAtlasiQ64446. baseline and differential.
GenevisibleiQ64446. MM.

Family and domain databases

CDDicd00371. HMA. 6 hits.
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.
IPR023214. HAD-like_dom.
IPR017969. Heavy-metal-associated_CS.
IPR006122. HMA_Cu_ion-bd.
IPR006121. HMA_dom.
IPR027256. P-typ_ATPase_IB.
IPR001757. P_typ_ATPase.
[Graphical view]
PfamiPF00122. E1-E2_ATPase. 1 hit.
PF00403. HMA. 6 hits.
[Graphical view]
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]
ProtoNetiSearch...

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: November 30, 2016
This is version 160 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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.