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UniProtKB - Q64446 (ATP7B_MOUSE)
Protein
Copper-transporting ATPase 2
Gene
Atp7b
Organism
Mus musculus (Mouse)
Status
Functioni
Copper ion transmembrane transporter involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
By similarityCatalytic activityi
- EC:7.2.2.8By similarity
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 79 | Cu(+) 1PROSITE-ProRule annotation | 1 | |
Metal bindingi | 82 | Cu(+) 1PROSITE-ProRule annotation | 1 | |
Metal bindingi | 164 | Cu(+) 2PROSITE-ProRule annotation | 1 | |
Metal bindingi | 167 | Cu(+) 2PROSITE-ProRule annotation | 1 | |
Metal bindingi | 278 | Cu(+) 3PROSITE-ProRule annotation | 1 | |
Metal bindingi | 281 | Cu(+) 3PROSITE-ProRule annotation | 1 | |
Metal bindingi | 372 | Cu(+) 4Curated | 1 | |
Metal bindingi | 501 | Cu(+) 5PROSITE-ProRule annotation | 1 | |
Metal bindingi | 504 | Cu(+) 5PROSITE-ProRule annotation | 1 | |
Metal bindingi | 577 | Cu(+) 6PROSITE-ProRule annotation | 1 | |
Metal bindingi | 580 | Cu(+) 6PROSITE-ProRule annotation | 1 | |
Active sitei | 1029 | 4-aspartylphosphate intermediateBy similarity | 1 | |
Metal bindingi | 1264 | Magnesium | 1 | |
Metal bindingi | 1268 | Magnesium | 1 |
GO - Molecular functioni
- ATP binding Source: HGNC-UCL
- ATP hydrolysis activity Source: InterPro
- copper ion binding Source: HGNC-UCL
- copper ion transmembrane transporter activity Source: MGI
- P-type divalent copper transporter activity Source: MGI
- P-type monovalent copper transporter activity Source: UniProtKB-EC
- zinc ion binding Source: MGI
GO - Biological processi
- cellular copper ion homeostasis Source: MGI
- cellular response to copper ion Source: MGI
- cellular zinc ion homeostasis Source: MGI
- copper ion export Source: MGI
- copper ion homeostasis Source: GO_Central
- copper ion import Source: MGI
- copper ion transport Source: MGI
- lactation Source: MGI
- protein maturation by copper ion transfer Source: MGI
- response to copper ion Source: MGI
- sequestering of calcium ion Source: MGI
Keywordsi
Molecular function | Translocase |
Biological process | Copper transport, Ion transport, Transport |
Ligand | ATP-binding, Copper, Magnesium, Metal-binding, Nucleotide-binding |
Enzyme and pathway databases
BRENDAi | 7.2.2.8, 3474 7.2.2.9, 3474 |
Reactomei | R-MMU-936837, Ion transport by P-type ATPases |
Names & Taxonomyi
Protein namesi | Recommended name: Copper-transporting ATPase 2 (EC:7.2.2.8By similarity)Alternative name(s): Copper pump 2 Wilson disease-associated protein homolog |
Gene namesi | Name:Atp7b Synonyms:Wnd |
Organismi | Mus musculus (Mouse) |
Taxonomic identifieri | 10090 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus |
Proteomesi |
|
Organism-specific databases
MGIi | MGI:103297, Atp7b |
VEuPathDBi | HostDB:ENSMUSG00000006567 |
Subcellular locationi
Endosome
- Late endosome By similarity
Golgi apparatus
- trans-Golgi network membrane By similarity; Multi-pass membrane protein Sequence analysis
Note: Predominantly found in the trans-Golgi network (TGN). Not redistributed to the plasma membrane in response to elevated copper levels.By similarity
Endoplasmic reticulum
- endoplasmic reticulum Source: MGI
Endosome
- late endosome Source: HGNC
Golgi apparatus
- Golgi apparatus Source: MGI
- trans-Golgi network Source: MGI
- trans-Golgi network membrane Source: UniProtKB
Plasma Membrane
- basolateral plasma membrane Source: MGI
- plasma membrane Source: MGI
Other locations
- apical part of cell Source: MGI
- bicellular tight junction Source: MGI
- integral component of membrane Source: UniProtKB-KW
- membrane Source: MGI
- perinuclear region of cytoplasm Source: MGI
- tight junction Source: MGI
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 1 – 655 | CytoplasmicSequence analysisAdd BLAST | 655 | |
Transmembranei | 656 – 677 | HelicalSequence analysisAdd BLAST | 22 | |
Topological domaini | 678 – 699 | ExtracellularSequence analysisAdd BLAST | 22 | |
Transmembranei | 700 – 719 | HelicalSequence analysisAdd BLAST | 20 | |
Topological domaini | 720 – 726 | CytoplasmicSequence analysis | 7 | |
Transmembranei | 727 – 747 | HelicalSequence analysisAdd BLAST | 21 | |
Topological domaini | 748 – 766 | ExtracellularSequence analysisAdd BLAST | 19 | |
Transmembranei | 767 – 787 | HelicalSequence analysisAdd BLAST | 21 | |
Topological domaini | 788 – 921 | CytoplasmicSequence analysisAdd BLAST | 134 | |
Transmembranei | 922 – 944 | HelicalSequence analysisAdd BLAST | 23 | |
Topological domaini | 945 – 974 | ExtracellularSequence analysisAdd BLAST | 30 | |
Transmembranei | 975 – 996 | HelicalSequence analysisAdd BLAST | 22 | |
Topological domaini | 997 – 1319 | CytoplasmicSequence analysisAdd BLAST | 323 | |
Transmembranei | 1320 – 1337 | HelicalSequence analysisAdd BLAST | 18 | |
Topological domaini | 1338 – 1348 | ExtracellularSequence analysisAdd BLAST | 11 | |
Transmembranei | 1349 – 1368 | HelicalSequence analysisAdd BLAST | 20 | |
Topological domaini | 1369 – 1462 | CytoplasmicSequence analysisAdd BLAST | 94 |
Keywords - Cellular componenti
Endosome, Golgi apparatus, MembranePathology & 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.1 Publication
Keywords - Diseasei
Disease variantPTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
ChainiPRO_0000046315 | 1 – 1462 | Copper-transporting ATPase 2Add BLAST | 1462 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 478 | PhosphoserineBy similarity | 1 | |
Modified residuei | 483 | PhosphoserineBy similarity | 1 | |
Modified residuei | 1395 | PhosphoserineBy similarity | 1 | |
Modified residuei | 1454 | PhosphoserineBy similarity | 1 |
Keywords - PTMi
PhosphoproteinProteomic databases
MaxQBi | Q64446 |
PaxDbi | Q64446 |
PRIDEi | Q64446 |
ProteomicsDBi | 277215 |
PTM databases
iPTMneti | Q64446 |
PhosphoSitePlusi | Q64446 |
Expressioni
Tissue specificityi
Detected in liver and kidney.
Gene expression databases
Bgeei | ENSMUSG00000006567, Expressed in stomach and 154 other tissues |
ExpressionAtlasi | Q64446, baseline and differential |
Genevisiblei | Q64446, 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 similarityProtein-protein interaction databases
BioGRIDi | 198269, 4 interactors |
STRINGi | 10090.ENSMUSP00000006742 |
Miscellaneous databases
RNActi | Q64446, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 68 – 134 | HMA 1PROSITE-ProRule annotationAdd BLAST | 67 | |
Domaini | 153 – 219 | HMA 2PROSITE-ProRule annotationAdd BLAST | 67 | |
Domaini | 267 – 333 | HMA 3PROSITE-ProRule annotationAdd BLAST | 67 | |
Domaini | 361 – 427 | HMA 4PROSITE-ProRule annotationAdd BLAST | 67 | |
Domaini | 490 – 556 | HMA 5PROSITE-ProRule annotationAdd BLAST | 67 | |
Domaini | 566 – 632 | HMA 6PROSITE-ProRule annotationAdd BLAST | 67 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 1 – 23 | DisorderedSequence analysisAdd BLAST | 23 | |
Regioni | 333 – 361 | DisorderedSequence analysisAdd BLAST | 29 | |
Regioni | 460 – 487 | DisorderedSequence analysisAdd BLAST | 28 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 343 – 361 | Polar residuesSequence analysisAdd BLAST | 19 | |
Compositional biasi | 471 – 487 | Polar residuesSequence analysisAdd BLAST | 17 |
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
Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily. [View classification]Curated
Keywords - Domaini
Repeat, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG0207, Eukaryota |
GeneTreei | ENSGT00940000155749 |
InParanoidi | Q64446 |
OMAi | NSWISGT |
OrthoDBi | 649559at2759 |
PhylomeDBi | Q64446 |
TreeFami | TF300460 |
Family and domain databases
CDDi | cd00371, HMA, 6 hits |
Gene3Di | 3.40.1110.10, 1 hit 3.40.50.1000, 1 hit |
InterProi | View protein in InterPro IPR023299, ATPase_P-typ_cyto_dom_N IPR018303, ATPase_P-typ_P_site IPR023298, ATPase_P-typ_TM_dom_sf IPR008250, ATPase_P-typ_transduc_dom_A_sf IPR036412, HAD-like_sf IPR023214, HAD_sf IPR017969, Heavy-metal-associated_CS IPR006122, HMA_Cu_ion-bd IPR006121, HMA_dom IPR036163, HMA_dom_sf IPR027256, P-typ_ATPase_IB IPR001757, P_typ_ATPase IPR044492, P_typ_ATPase_HD_dom |
Pfami | View protein in Pfam PF00403, HMA, 6 hits |
SFLDi | SFLDF00027, p-type_atpase, 1 hit |
SUPFAMi | SSF55008, SSF55008, 6 hits SSF56784, SSF56784, 1 hit SSF81653, SSF81653, 1 hit SSF81665, SSF81665, 1 hit |
TIGRFAMsi | TIGR01525, ATPase-IB_hvy, 1 hit TIGR01494, ATPase_P-type, 2 hits TIGR00003, TIGR00003, 5 hits |
PROSITEi | View protein in PROSITE PS00154, ATPASE_E1_E2, 1 hit PS01047, HMA_1, 5 hits PS50846, HMA_2, 6 hits |
(1+)i Sequence
Sequence statusi: Complete.
This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All
Q64446-1 [UniParc]FASTAAdd to basket
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
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketB1AQ57 | B1AQ57_MOUSE | P-type Cu(+) transporter | Atp7b | 1,347 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 107 – 108 | SA → KH in AAC52852 (PubMed:8894697).Curated | 2 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural varianti | 1356 | M → V in tx mice. | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | U38477 mRNA Translation: AAC52852.1 AC163439 Genomic DNA No translation available. AL590619 Genomic DNA No translation available. CH466580 Genomic DNA Translation: EDL32910.1 |
CCDSi | CCDS22168.1 |
RefSeqi | NP_031537.2, NM_007511.2 |
Genome annotation databases
Ensembli | ENSMUST00000006742; ENSMUSP00000006742; ENSMUSG00000006567 |
GeneIDi | 11979 |
KEGGi | mmu:11979 |
UCSCi | uc009lck.1, mouse |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | U38477 mRNA Translation: AAC52852.1 AC163439 Genomic DNA No translation available. AL590619 Genomic DNA No translation available. CH466580 Genomic DNA Translation: EDL32910.1 |
CCDSi | CCDS22168.1 |
RefSeqi | NP_031537.2, NM_007511.2 |
3D structure databases
AlphaFoldDBi | Q64446 |
SMRi | Q64446 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 198269, 4 interactors |
STRINGi | 10090.ENSMUSP00000006742 |
PTM databases
iPTMneti | Q64446 |
PhosphoSitePlusi | Q64446 |
Proteomic databases
MaxQBi | Q64446 |
PaxDbi | Q64446 |
PRIDEi | Q64446 |
ProteomicsDBi | 277215 |
Protocols and materials databases
Antibodypediai | 2396, 530 antibodies from 36 providers |
DNASUi | 11979 |
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 |
VEuPathDBi | HostDB:ENSMUSG00000006567 |
Phylogenomic databases
eggNOGi | KOG0207, Eukaryota |
GeneTreei | ENSGT00940000155749 |
InParanoidi | Q64446 |
OMAi | NSWISGT |
OrthoDBi | 649559at2759 |
PhylomeDBi | Q64446 |
TreeFami | TF300460 |
Enzyme and pathway databases
BRENDAi | 7.2.2.8, 3474 7.2.2.9, 3474 |
Reactomei | R-MMU-936837, Ion transport by P-type ATPases |
Miscellaneous databases
BioGRID-ORCSi | 11979, 3 hits in 71 CRISPR screens |
PROi | PR:Q64446 |
RNActi | Q64446, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSMUSG00000006567, Expressed in stomach and 154 other tissues |
ExpressionAtlasi | Q64446, baseline and differential |
Genevisiblei | Q64446, MM |
Family and domain databases
CDDi | cd00371, HMA, 6 hits |
Gene3Di | 3.40.1110.10, 1 hit 3.40.50.1000, 1 hit |
InterProi | View protein in InterPro IPR023299, ATPase_P-typ_cyto_dom_N IPR018303, ATPase_P-typ_P_site IPR023298, ATPase_P-typ_TM_dom_sf IPR008250, ATPase_P-typ_transduc_dom_A_sf IPR036412, HAD-like_sf IPR023214, HAD_sf IPR017969, Heavy-metal-associated_CS IPR006122, HMA_Cu_ion-bd IPR006121, HMA_dom IPR036163, HMA_dom_sf IPR027256, P-typ_ATPase_IB IPR001757, P_typ_ATPase IPR044492, P_typ_ATPase_HD_dom |
Pfami | View protein in Pfam PF00403, HMA, 6 hits |
SFLDi | SFLDF00027, p-type_atpase, 1 hit |
SUPFAMi | SSF55008, SSF55008, 6 hits SSF56784, SSF56784, 1 hit SSF81653, SSF81653, 1 hit SSF81665, SSF81665, 1 hit |
TIGRFAMsi | TIGR01525, ATPase-IB_hvy, 1 hit TIGR01494, ATPase_P-type, 2 hits TIGR00003, TIGR00003, 5 hits |
PROSITEi | View protein in PROSITE PS00154, ATPASE_E1_E2, 1 hit PS01047, HMA_1, 5 hits PS50846, HMA_2, 6 hits |
MobiDBi | Search... |
Entry informationi
Entry namei | ATP7B_MOUSE | |
Accessioni | Q64446Primary (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: | May 25, 2022 | |
This is version 193 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families