Copper-transporting ATPase 2 - Mus musculus (Mouse)

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Q64446 (ATP7B_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified May 1, 2013. Version 126. History...

Clusters with 100%, 90%, 50% identity |

Documents (2) |

Third-party data

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Names·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·Documents Customize order

Names and origin

Protein names

Recommended name:
Copper-transporting ATPase 2
EC=3.6.3.4

Alternative name(s):
Copper pump 2
Wilson disease-associated protein homolog

Gene names

Name:	Atp7b
Synonyms:	Wnd

Organism

Mus musculus (Mouse) [Reference proteome]

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus › Mus

Protein attributes

Sequence length	1462 AA.
Sequence status	Complete.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile By similarity.
Catalytic activity	ATP + H₂O + Cu²⁺(In) = ADP + phosphate + Cu²⁺(Out).
Subunit structure	Monomer. Interacts with COMMD1/MURR1 By similarity. Interacts with DCTN4, in a copper-dependent manner. Interacts with ATOX1 By similarity.
Subcellular location	Golgi apparatus › trans-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.
Tissue specificity	Detected in liver and kidney.
Domain	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.
Involvement in disease	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.
Sequence similarities	Belongs to the cation transport ATPase (P-type) (TC 3.A.3) family. Type IB subfamily. [View classification] Contains 6 HMA domains.

Ontologies

Keywords
Biological process	Copper transport Ion transport Transport
Cellular component	Golgi apparatus Membrane
Disease	Disease mutation
Domain	Repeat Transmembrane Transmembrane helix
Ligand	ATP-binding Copper Magnesium Metal-binding Nucleotide-binding
Molecular function	Hydrolase
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	cellular copper ion homeostasis Inferred from mutant phenotype PubMed 10441329 PubMed 11085952 PubMed 1588441 PubMed 2845190 PubMed 6863890 PubMed 8040371 PubMed 8257436 PubMed 9392450 PubMed 9484715. Source: MGI cellular zinc ion homeostasis Inferred from mutant phenotype PubMed 8040371 PubMed 8257436 PubMed 9392450 PubMed 9484715. Source: MGI copper ion import Inferred from electronic annotation. Source: Compara intracellular copper ion transport Inferred from mutant phenotype PubMed 16436657. Source: MGI lactation Inferred from mutant phenotype PubMed 1588441 PubMed 6863890. Source: MGI response to copper ion Inferred from electronic annotation. Source: Compara sequestering of calcium ion Inferred from electronic annotation. Source: Compara
Cellular_component	Golgi apparatus Inferred from electronic annotation. Source: UniProtKB-SubCell basolateral plasma membrane Inferred from electronic annotation. Source: Compara cytoplasmic membrane-bounded vesicle Inferred from electronic annotation. Source: Compara integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW late endosome Inferred from sequence or structural similarity. Source: UniProtKB membrane Inferred from direct assay PubMed 10441329 PubMed 15634671. Source: MGI perinuclear region of cytoplasm Inferred from electronic annotation. Source: Compara trans-Golgi network Inferred from direct assay PubMed 11085952. Source: MGI
Molecular_function	ATP binding Inferred from sequence or structural similarity. Source: HGNC copper ion binding Inferred from sequence or structural similarity. Source: HGNC copper-exporting ATPase activity Inferred from direct assay PubMed 15634671. Source: MGI
Complete GO annotation...

Sequence annotation (Features)

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Molecule processing

Chain

1 – 1462

1462

Copper-transporting ATPase 2

PRO_0000046315

Regions

Topological domain

1 – 655

655

Cytoplasmic Potential

Transmembrane

656 – 677

Helical; Potential

Topological domain

678 – 699

Extracellular Potential

Transmembrane

700 – 719

Helical; Potential

Topological domain

720 – 726

Cytoplasmic Potential

Transmembrane

727 – 747

Helical; Potential

Topological domain

748 – 766

Extracellular Potential

Transmembrane

767 – 787

Helical; Potential

Topological domain

788 – 921

134

Cytoplasmic Potential

Transmembrane

922 – 944

Helical; Potential

Topological domain

945 – 974

Extracellular Potential

Transmembrane

975 – 996

Helical; Potential

Topological domain

997 – 1319

323

Cytoplasmic Potential

Transmembrane

1320 – 1337

Helical; Potential

Topological domain

1338 – 1348

Extracellular Potential

Transmembrane

1349 – 1368

Helical; Potential

Topological domain

1369 – 1462

Cytoplasmic Potential

Domain

69 – 135

HMA 1

Domain

154 – 220

HMA 2

Domain

268 – 334

HMA 3

Domain

362 – 428

HMA 4

Domain

491 – 557

HMA 5

Domain

567 – 633

HMA 6

Sites

Active site

1029

4-aspartylphosphate intermediate By similarity

Metal binding

Copper 1 By similarity

Metal binding

Copper 1 By similarity

Metal binding

164

Copper 2 By similarity

Metal binding

167

Copper 2 By similarity

Metal binding

278

Copper 3 By similarity

Metal binding

281

Copper 3 By similarity

Metal binding

372

Copper 4 By similarity

Metal binding

501

Copper 5 By similarity

Metal binding

504

Copper 5 By similarity

Metal binding

577

Copper 6 By similarity

Metal binding

580

Copper 6 By similarity

Metal binding

1264

Magnesium By similarity

Metal binding

1268

Magnesium By similarity

Natural variations

Natural variant

1356

M → V in tx mice.

Experimental info

Sequence conflict

107 – 108

SA → KH in AAC52852. Ref.1

Sequences

Sequence

Length

Mass (Da)

Tools

Q64446 [UniParc].

Last modified July 27, 2011. Version 2.
Checksum: BEC219F80EB74214
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FASTA

1,462

157,190

        10         20         30         40         50         60 
MDPRKNLASV GTMPEQERQV TAKEASRKIL SKLALPGRPW EQSMKQSFAF DNVGYEGGLD 

        70         80         90        100        110        120 
STSSSPAATD VVNILGMTCH SCVKSIEDRI SSLKGIVNIK VSLEQGSATV RYVPSVMNLQ 

       130        140        150        160        170        180 
QICLQIEDMG FEASAAEGKA ASWPSRSSPA QEAVVKLRVE GMTCQSCVSS IEGKIRKLQG 

       190        200        210        220        230        240 
VVRIKVSLSN QEAVITYQPY LIQPEDLRDH ICDMGFEAAI KNRTAPLRLG PIDVNKLEST 

       250        260        270        280        290        300 
NLKKETVSPV QISNHFETLG HQGSYLATLP LRIDGMHCKS CVLNIEGNIG QLPGVQNIHV 

       310        320        330        340        350        360 
SLENKTAQIQ YDPSCVTPMF LQTAIEALPP GHFKVSLPDG VEENEPQSGS SQRHQEQGPG 

       370        380        390        400        410        420 
RTAVLTISGI TCASSVQPIE DMLSQRKGVQ QTSISLAEGT GAVLYDPSIV SLDELRTAVE 

       430        440        450        460        470        480 
DMGFEVSVNS ETFTINPVRN FKSGNSVPQT MGDIAGSVQK MAPDTRGLPT HQGPGHSSET 

       490        500        510        520        530        540 
PSSPGATASQ KCFVQIKGMT CASCVSNIER SLQRHAGILS VLVALMSGKA EVKYDPEIIQ 

       550        560        570        580        590        600 
SPRIAQLIQD LGFEASVMED NTVSEGDIEL IITGMTCASC VHNIESKLTR TNGITYASVA 

       610        620        630        640        650        660 
LATSKAHVKF DPEIVGPRDI IKIIEEIGFH ASLAQRNPNA HHLDHKTEIK QWKKSFLCSL 

       670        680        690        700        710        720 
VFGIPVMGLM VYMLIPSSTP QETMVLDHNI IPGLSVLNLI FFILCTFVQF LGGWYFYVQA 

       730        740        750        760        770        780 
YKSLRHRSAN MDVLIVLATT IAYAYSLVIL VVAVAEKAEK SPVTFFDTPP MLFVFIALGR 

       790        800        810        820        830        840 
WLEHVAKSKT SEALAKLMSL QATEATVVTL GEDNLILREE QVPMELVQRG DVIKVVPGGK 

       850        860        870        880        890        900 
FPVDGKVLEG NTMADESLIT GEAMPVTKKP GSIVIAGSIN AHGSVLLKAT HVGNDTTLAQ 

       910        920        930        940        950        960 
IVKLVEEAQM SKAPIQQLAD RFSGYFVPFI IIISTLTLVV WIVIGFVDFG VVQKYFPSPS 

       970        980        990       1000       1010       1020 
KHISQTEVII RFAFQTSITV LCIACPCSLG LATPTAVMVG TGVAAQNGVL IKGGKPLEMA 

      1030       1040       1050       1060       1070       1080 
HKIKTVMFDK TGTITHGVPR VMRFLLLADV ATLPLRKVLA VVGTAEASSE HPLGVAVTKY 

      1090       1100       1110       1120       1130       1140 
CKEELGTETL GYSTDFQAVP GCGISCKVSN VEGILARSDL TAHPVGVGNP PTGEGAGPQT 

      1150       1160       1170       1180       1190       1200 
FSVLIGNREW MRRNGLTISS DISDAMTDHE MKGQTAILVA IDGVLCGMIA IADAVKPEAA 

      1210       1220       1230       1240       1250       1260 
LAIYTLKSMG VDVALITGDN RKTARAIATQ VGINKVFAEV LPSHKVAKVQ ELQNEGKKVA 

      1270       1280       1290       1300       1310       1320 
MVGDGVNDSP ALAQADVGIA IGTGTDVAIE AADVVLIRND LLDVVASIHL SKRTVRRIRV 

      1330       1340       1350       1360       1370       1380 
NLVLALIYNM VGIPIAAGVF MPIGIVLQPW MGSAAMAASS VSVVLSSLQL KCYRKPDLER 

      1390       1400       1410       1420       1430       1440 
YEAQAHGRMK PLSASQVSVH IGMDDRRRDS PRATAWDQVS YVSQVSLSSL TSDRLSRHGG 

      1450       1460 
AAEDGGDKWS LLLSDRDEEQ CI

« Hide

References

	« Hide 'large scale' referencesShow 'large scale' references »
[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]	"Lineage-specific biology revealed by a finished genome assembly of the mouse." Church D.M., Goodstadt L., Hillier L.W., Zody M.C., Goldstein S., She X., Bult C.J., Agarwala R., Cherry J.L., DiCuccio M., Hlavina W., Kapustin Y., Meric P., Maglott D., Birtle Z., Marques A.C., Graves T., Zhou S. Ponting C.P. PLoS Biol. 7:E1000112-E1000112(2009) [PubMed] [Europe PMC] [Abstract] 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].
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	U38477 mRNA. Translation: AAC52852.1. AL590619, AC163439 Genomic DNA. Translation: CAM22052.1. CH466580 Genomic DNA. Translation: EDL32910.1.
IPI	IPI00134479.
RefSeq	NP_031537.2. NM_007511.2.
UniGene	Mm.87854.
3D structure databases
ProteinModelPortal	Q64446.
SMR	Q64446. Positions 68-1378.
ModBase	Search...
Protein-protein interaction databases
STRING	10090.ENSMUSP00000006742.
PTM databases
PhosphoSite	Q64446.
Proteomic databases
PaxDb	Q64446.
PRIDE	Q64446.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENSMUST00000006742; ENSMUSP00000006742; ENSMUSG00000006567.
GeneID	11979.
KEGG	mmu:11979.
Organism-specific databases
CTD	540.
MGI	MGI:103297. Atp7b.
Phylogenomic databases
eggNOG	COG2217.
GeneTree	ENSGT00530000063773.
HOGENOM	HOG000250397.
HOVERGEN	HBG050616.
InParanoid	B1AQ56.
KO	K01533.
OMA	QIKGMTC.
OrthoDB	EOG43N7BZ.
Enzyme and pathway databases
BRENDA	3.6.3.4. 2681.
Gene expression databases
ArrayExpress	Q64446.
Bgee	Q64446.
CleanEx	MM_ATP7B.
Genevestigator	Q64446.
GermOnline	ENSMUSG00000006567. Mus musculus.
Family and domain databases
Gene3D	2.70.150.10. 1 hit. 3.40.1110.10. 1 hit. 3.40.50.1000. 2 hits.
InterPro	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]
PANTHER	PTHR24093. PTHR24093. 1 hit.
Pfam	PF00122. E1-E2_ATPase. 1 hit. PF00403. HMA. 6 hits. PF00702. Hydrolase. 1 hit. [Graphical view]
PRINTS	PR00119. CATATPASE.
SUPFAM	SSF56784. HAD-like_dom. 1 hit. SSF55008. HeavyMe_transpt. 6 hits.
TIGRFAMs	TIGR01525. ATPase-IB_hvy. 1 hit. TIGR01494. ATPase_P-type. 2 hits. TIGR00003. TIGR00003. 5 hits.
PROSITE	PS00154. ATPASE_E1_E2. 1 hit. PS01047. HMA_1. 5 hits. PS50846. HMA_2. 6 hits. [Graphical view]
ProtoNet	Search...
Other
NextBio	280115.
SOURCE	Search...

Entry information

Entry name

ATP7B_MOUSE

Accession

Primary (citable) accession number: Q64446
Secondary accession number(s): B1AQ56

Entry history

Integrated into UniProtKB/Swiss-Prot:	December 8, 2000
Last sequence update:	July 27, 2011
Last modified:	May 1, 2013
This is version 126 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Relevant documents

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot

SIMILARITY comments

Index of protein domains and families

Names·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·Documents

Preferred order of sections

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Sequence annotation (Features)

Molecule processing

Regions

Sites

Natural variations

Experimental info

Sequences

References

Cross-references

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Gene expression databases

Family and domain databases

Other

Entry information

Relevant documents