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Q64446

- ATP7B_MOUSE

UniProt

Q64446 - ATP7B_MOUSE

Protein

Copper-transporting ATPase 2

Gene

Atp7b

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 139 (01 Oct 2014)
      Sequence version 2 (27 Jul 2011)
      Previous versions | rss
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    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

    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

    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.
    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, 647-1376.
    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.
    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

    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, 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

    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 1, 2014
    This is version 139 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