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Q64446

- ATP7B_MOUSE

UniProt

Q64446 - ATP7B_MOUSE

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Protein

Copper-transporting ATPase 2

Gene

Atp7b

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 1PROSITE-ProRule annotation
Metal bindingi82 – 821Copper 1PROSITE-ProRule annotation
Metal bindingi164 – 1641Copper 2PROSITE-ProRule annotation
Metal bindingi167 – 1671Copper 2PROSITE-ProRule annotation
Metal bindingi278 – 2781Copper 3PROSITE-ProRule annotation
Metal bindingi281 – 2811Copper 3PROSITE-ProRule annotation
Metal bindingi372 – 3721Copper 4PROSITE-ProRule annotation
Metal bindingi501 – 5011Copper 5PROSITE-ProRule annotation
Metal bindingi504 – 5041Copper 5PROSITE-ProRule annotation
Metal bindingi577 – 5771Copper 6PROSITE-ProRule annotation
Metal bindingi580 – 5801Copper 6PROSITE-ProRule annotation
Active sitei1029 – 102914-aspartylphosphate intermediateBy similarity
Metal bindingi1264 – 12641MagnesiumPROSITE-ProRule annotation
Metal bindingi1268 – 12681MagnesiumPROSITE-ProRule annotation

GO - Molecular functioni

  1. ATP binding Source: HGNC
  2. copper-exporting ATPase activity Source: MGI
  3. copper ion binding Source: HGNC
  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 transmembrane transport Source: GOC
  6. copper ion transport Source: MGI
  7. intracellular copper ion transport Source: MGI
  8. lactation Source: MGI
  9. response to copper ion Source: Ensembl
  10. 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 By similarity; 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).By similarity

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 2PRO_0000046315Add
BLAST

Proteomic databases

MaxQBiQ64446.
PaxDbiQ64446.
PRIDEiQ64446.

PTM databases

PhosphoSiteiQ64446.

Expressioni

Tissue specificityi

Detected in liver and kidney.

Gene expression databases

BgeeiQ64446.
CleanExiMM_ATP7B.
ExpressionAtlasiQ64446. baseline and differential.
GenevestigatoriQ64446.

Interactioni

Subunit structurei

Monomer. Interacts with COMMD1/MURR1 (By similarity). Interacts with DCTN4, in a copper-dependent manner. Interacts with ATOX1 (By similarity).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, 710-1376.
ModBaseiSearch...
MobiDBiSearch...

Topological domain

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 655655CytoplasmicSequence AnalysisAdd
BLAST
Topological domaini678 – 69922ExtracellularSequence AnalysisAdd
BLAST
Topological domaini720 – 7267CytoplasmicSequence Analysis
Topological domaini748 – 76619ExtracellularSequence AnalysisAdd
BLAST
Topological domaini788 – 921134CytoplasmicSequence AnalysisAdd
BLAST
Topological domaini945 – 97430ExtracellularSequence AnalysisAdd
BLAST
Topological domaini997 – 1319323CytoplasmicSequence AnalysisAdd
BLAST
Topological domaini1338 – 134811ExtracellularSequence AnalysisAdd
BLAST
Topological domaini1369 – 146294CytoplasmicSequence AnalysisAdd
BLAST

Transmembrane

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei656 – 67722HelicalSequence AnalysisAdd
BLAST
Transmembranei700 – 71920HelicalSequence AnalysisAdd
BLAST
Transmembranei727 – 74721HelicalSequence AnalysisAdd
BLAST
Transmembranei767 – 78721HelicalSequence AnalysisAdd
BLAST
Transmembranei922 – 94423HelicalSequence AnalysisAdd
BLAST
Transmembranei975 – 99622HelicalSequence AnalysisAdd
BLAST
Transmembranei1320 – 133718HelicalSequence AnalysisAdd
BLAST
Transmembranei1349 – 136820HelicalSequence AnalysisAdd
BLAST

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini69 – 13567HMA 1PROSITE-ProRule annotationAdd
BLAST
Domaini154 – 22067HMA 2PROSITE-ProRule annotationAdd
BLAST
Domaini268 – 33467HMA 3PROSITE-ProRule annotationAdd
BLAST
Domaini362 – 42867HMA 4PROSITE-ProRule annotationAdd
BLAST
Domaini491 – 55767HMA 5PROSITE-ProRule annotationAdd
BLAST
Domaini567 – 63367HMA 6PROSITE-ProRule annotationAdd
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).By similarity

Sequence similaritiesi

Contains 6 HMA domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiCOG2217.
GeneTreeiENSGT00530000063773.
HOGENOMiHOG000250397.
HOVERGENiHBG050616.
InParanoidiQ64446.
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

        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)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti107 – 1082SA → KH in AAC52852. (PubMed:8894697)Curated

Natural variant

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

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, 710-1376.
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 Q64446.
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.
ExpressionAtlasi Q64446. baseline and differential.
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: October 29, 2014
This is version 140 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