[1]	NUCLEOTIDE SEQUENCE [MRNA]. STRAIN=Sprague-Dawley; TISSUE=Liver; DOI=10.1038/ng0894-541; PubMed=7951327 [NCBI, ExPASy, EBI, Israel, Japan] Wu J., Forbes J.R., Chen H.S., Cox D.W.; "The LEC rat has a deletion in the copper transporting ATPase gene homologous to the Wilson disease gene."; Nat. Genet. 7:541-545(1994).
[2]	NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM SHORT). STRAIN=Sprague-Dawley; TISSUE=Pineal gland; PubMed=9920665 [NCBI, ExPASy, EBI, Israel, Japan] Borjigin J., Payne A.S., Deng J., Li X., Wang M.M., Ovodenko B., Gitlin J.D., Snyder S.H.; "A novel pineal night-specific ATPase encoded by the Wilson disease gene."; J. Neurosci. 19:1018-1026(1999).
[3]	NUCLEOTIDE SEQUENCE [MRNA] OF 530-616. STRAIN=Sprague-Dawley; TISSUE=Liver; PubMed=8037655 [NCBI, ExPASy, EBI, Israel, Japan] Yamaguchi Y., Heiny M.E., Shimizu N., Aoki T., Gitlin J.D.; "Expression of the Wilson disease gene is deficient in the Long-Evans Cinnamon rat."; Biochem. J. 301:1-4(1994).

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: Monomer. Interacts with COMMD1/MURR1 (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).

ALTERNATIVE PRODUCTS: 3 named isoforms [FASTA] produced by alternative splicing and alternative initiation.

Name	Long
Isoform ID	Q64535-1
This is the isoform sequence displayed in this entry.

Name	Short
Isoform ID	Q64535-2
Note: Produced by alternative splicing. Does not show copper transport activity.
Features which should be applied to build the isoform sequence: VSP_000428.

Name	PINAM2
Isoform ID	Q64535-3
Note: Produced by alternative initiation at Met-815 of isoform Long. Shows copper transport activity.
Features which should be applied to build the isoform sequence: VSP_018666.

TISSUE SPECIFICITY: Expressed in brain, liver, kidney, spleen and stomach. In brain, detected in neuronal cells of the hippocampal formation, olfactory bulbs, cerebellum, cerebral cortex and nuclei in the brainstem. Isoform PINA is expressed during night in adult pineal gland (pinealocytes) and retina. Isoform PINA is not detected in other tissue.
DEVELOPMENTAL STAGE: Isoform PINA is expressed during daytime in embryonic pineal (postnatal day 2 and 7) and embryonic retinal pigment epithelium (embryonic day 14.5 and postnatal day 16). Daytime expression disappears in pineal at postnatal day 16 and in adult retina.
DISEASE: Deficiency of Atp7b expression is the cause of the Long-Evans Cinnamon (LEC) phenotype, inherited in an autosomal recessive manner, characterized by excessive hepatic copper accumulation, defective holoceruloplasmin biosynthesis, impaired biliary copper excretion and the development of necrotizing hepatitis by 4 months of age.
SIMILARITY: Belongs to the cation transport ATPase (P-type) family. Type IB subfamily.
SIMILARITY: Contains 6 HMA domains.

Copyright

Copyrighted by the UniProt Consortium, see http://www.uniprot.org/terms. Distributed under the Creative Commons Attribution-NoDerivs License.

Cross-references

Sequence databases

EMBL

U08344; AAA62157.1; -; mRNA.	[EMBL / GenBank / DDBJ] [CoDingSequence]
AF120492; AAD16009.1; -; mRNA.	[EMBL / GenBank / DDBJ] [CoDingSequence]
L28173; AAA21810.1; -; mRNA.	[EMBL / GenBank / DDBJ] [CoDingSequence]

PIR

I58124; I58124.

UniGene

Rn.10025

3D structure databases

HSSP

Q04656; 1AW0. [HSSP ENTRY / PDB]

SMR

Q64535; 478-550, 479-626, 1027-1184.

ModBase

Q64535.

Organism-specific databases

RGD

2180; Atp7b.

Gene expression databases

ArrayExpress

Q64535; -.

GermOnline

ENSRNOG00000012878; Rattus norvegicus.

Family and domain databases

InterPro

IPR006416; ATPase-IB_hvy.
IPR001757; ATPase_P.
IPR006403; ATPase_P_cat/Cu.
IPR001877; Cu_ATPase1.
IPR006122; Cu_ion_bd.
IPR005834; Dehalogen-like_hydro.
IPR008250; E1-E2_ATPase_reg.
IPR006121; HeavyMe_transpt.
Graphical view of domain structure.

PANTHER

PTHR11939; ATPase_P; 1.

Pfam

PF00122; E1-E2_ATPase; 1.
PF00403; HMA; 6.
PF00702; Hydrolase; 1.
Pfam graphical view of domain structure.

PRINTS

PR00119; CATATPASE.
PR00942; CUATPASEI.

TIGRFAMs

TIGR01511; ATPase-IB1_Cu; 1.
TIGR01525; ATPase-IB_hvy; 1.
TIGR01494; ATPase_P-type; 2.
TIGR00003; Cu_ion_bd; 2.

PROSITE

PS00154; ATPASE_E1_E2; 1.
PS01047; HMA_1; 5.
PS50846; HMA_2; 6.
PROSITE graphical view of domain structure (profiles).

BLOCKS

Q64535.

Genome annotation databases

Ensembl

ENSRNOG00000012878; Rattus norvegicus. [Contig view]

Phylogenomic databases

HOVERGEN

Q64535; -.

Other

ProtoNet

Q64535.

UniRef

View cluster of proteins with at least 50% / 90% / 100% identity.

Keywords

Alternative initiation; Alternative splicing; ATP-binding; Biological rhythms; Copper; Copper transport; Golgi apparatus; Hydrolase; Ion transport; Magnesium; Membrane; Metal-binding; Nucleotide-binding; Phosphoprotein; Repeat; Transmembrane; Transport.

Features

Feature table viewer

Feature aligner

`Key`	`From To`	`Length`	`Description`	`FTId`
`CHAIN`	`1 1451`	`1451`	`Copper-transporting ATPase 2.`	`PRO_0000002511`
`TOPO_DOM`	`1 646`	`646`	`Cytoplasmic (Potential).`
`TRANSMEM`	`647 668`	`22`	`Potential.`
`TOPO_DOM`	`669 690`	`22`	`Extracellular (Potential).`
`TRANSMEM`	`691 710`	`20`	`Potential.`
`TOPO_DOM`	`711 717`	`7`	`Cytoplasmic (Potential).`
`TRANSMEM`	`718 738`	`21`	`Potential.`
`TOPO_DOM`	`739 757`	`19`	`Extracellular (Potential).`
`TRANSMEM`	`758 778`	`21`	`Potential.`
`TOPO_DOM`	`779 912`	`134`	`Cytoplasmic (Potential).`
`TRANSMEM`	`913 935`	`23`	`Potential.`
`TOPO_DOM`	`936 965`	`30`	`Extracellular (Potential).`
`TRANSMEM`	`966 987`	`22`	`Potential.`
`TOPO_DOM`	`988 1310`	`323`	`Cytoplasmic (Potential).`
`TRANSMEM`	`1311 1328`	`18`	`Potential.`
`TOPO_DOM`	`1329 1339`	`11`	`Extracellular (Potential).`
`TRANSMEM`	`1340 1357`	`18`	`Potential.`
`TOPO_DOM`	`1358 1451`	`94`	`Cytoplasmic (Potential).`
`DOMAIN`	`58 124`	`67`	`HMA 1.`
`DOMAIN`	`143 209`	`67`	`HMA 2.`
`DOMAIN`	`257 323`	`67`	`HMA 3.`
`DOMAIN`	`356 422`	`67`	`HMA 4.`
`DOMAIN`	`482 548`	`67`	`HMA 5.`
`DOMAIN`	`558 624`	`67`	`HMA 6.`
`COMPBIAS`	`921 924`	`4`	`Poly-Ile.`
`ACT_SITE`	`1020 1020`		`4-aspartylphosphate intermediate (By similarity).`
`METAL`	`1255 1255`		`Magnesium (By similarity).`
`METAL`	`1259 1259`		`Magnesium (By similarity).`
`VAR_SEQ`	`1 814`		`Missing (in isoform PINAM2).`	`VSP_018666`
`VAR_SEQ`	`1 788`		`Missing (in isoform Short).`	`VSP_000428`
`CONFLICT`	`1194 1195`		`SI -> IY (in Ref. 2; AAD16009).`
`CONFLICT`	`1281 1281`		`D -> E (in Ref. 2; AAD16009).`
`CONFLICT`	`1346 1346`		`A -> AMA (in Ref. 2).`

Sequence information

Length: 1451 AA [This is the length of the unprocessed precursor]

Molecular weight: 155990 Da [This is the MW of the unprocessed precursor]

CRC64: 2029940643318401 [This is a checksum on the sequence]

        10         20         30         40         50         60 
MPEQERKVTA KEASRKILSK LALPTRPWGQ SMKQSFAFDN VGYEGGLDST CFILQLTTGV 

        70         80         90        100        110        120 
VSILGMTCHS CVKSIEDRIS SLKGIVSIKV SLEQGSATVK YVPSVLNLQQ ICLQIEDMGF 

       130        140        150        160        170        180 
EASAAEGKAA SWPSRSSPAQ EAVVKLRVEG MTCQSCVSSI EGKIRKLQGV VRVKVSLSNQ 

       190        200        210        220        230        240 
EAVITYQPYL IQPEDLRDHI CDMGFEAAIK NRTAPLRLGP IDINKLESTN LKRAAVPPIQ 

       250        260        270        280        290        300 
NSNHLETPGH QQNHLATLPL RIDGMHCKSC VLNIEGNIGQ LPGVQNIHVS LENKTAQVQY 

       310        320        330        340        350        360 
DSSCITPLFL QTAIEALPPG YFKVSLPDGL EKESGSSSVP SLGSSQRQQE PGPCRTAVLT 

       370        380        390        400        410        420 
ITGIPRDSSV QPMEDMLSQM KGVQQIDISL AEGTGAVLYD PSVVSSDELR TAVEDMGFEV 

       430        440        450        460        470        480 
SVNPENITTN RVSSGNSVPQ AVGDSPGSVQ NMASDTRGLL THQGPGYLSD SPPSPGGTAS 

       490        500        510        520        530        540 
QKCFVQIKGM TCASCVSNIE RSLQRHAGIL SVLVALMSGK AEVKYDPEVI QSPRIAQLIE 

       550        560        570        580        590        600 
DLGFEAAIME DNTVSEGDIE LIITGMTCAS CVHNIESKLT RTNGITYASV ALATSKAHVK 

       610        620        630        640        650        660 
FDPEIIGPRD IIKVIEEIGF HASLAHRNPN AHHLDHKTEI KQWKKSFLCS LVFGIPVMGL 

       670        680        690        700        710        720 
MIYMLIPSSK PHETMVLDHN IIPGLSVLNL IFFILCTFVQ FLGGWYFYVQ AYKSLRHKSA 

       730        740        750        760        770        780 
NMDVLIVLAT TIAYAYSLVI LVVAIAEKAE KSPVTFFDTP PMLFVFIALG RWLEHVAKSK 

       790        800        810        820        830        840 
TSEALAKLMS LQATEATVVT LGEDNLILRE EQVPMELVQR GDIIKVVPGG KFPVDGKVLE 

       850        860        870        880        890        900 
GNTMADESLI TGEAMPVTKK PGSIVIAGSI NAHGSVLIKA THVGNDTTLA QIVKLVEEAQ 

       910        920        930        940        950        960 
MSKAPIQQLA DRFSGYFVPF IIIISTLTLV VWIIIGFVDF GIVQKYFPSP SKHISQTEVI 

       970        980        990       1000       1010       1020 
IRFAFQTSIT VLCIACPCSL GLATPTAVMV GTGVAAQNGV LIKGGKPLEM AHKIKTVMFD 

      1030       1040       1050       1060       1070       1080 
KTGTITHGVP RVMRFLLLVD VATLSLRKVL AVVGTAEASS EHPLGVAVTK YCKEELGTET 

      1090       1100       1110       1120       1130       1140 
LGYSTDFQAV PGCGISCKVS NVESILAHRG PTAHPIGVGN PPIGEGTGPQ TFSVLIGNRE 

      1150       1160       1170       1180       1190       1200 
WMRRNGLTIS SDISDAMTDH EMKGQTAILV AIDGVLCGMI AIADAVKPEA ALASITLKSM 

      1210       1220       1230       1240       1250       1260 
GVDVALITGD NRKTARAIAT QVGINKVFAE VLPSHKVAKV QELQNKGKKV AMVGDGVNDS 

      1270       1280       1290       1300       1310       1320 
PALAQADVGI AIGTGTDVAI DAADVVLIRN DLLDVVASIH LSKRTVRRIR VNLVLALIYN 

      1330       1340       1350       1360       1370       1380 
MVGIPIAAGV FMPIGIVLQP WMGSAAASSV SVVLSSLQLK CYRKPDLERY EAQAHGRMKP 

      1390       1400       1410       1420       1430       1440 
LSASQVSVHV GMDDRRRDSP RATPWDQVSY VSQVSLSSLT SDRLSRHGGM AEDGGDKWSL 

      1450 
LLSDRDEEQC I

Q64535 in FASTA format

View entry in original UniProtKB/Swiss-Prot format
View entry in raw text format (no links)
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	BLAST submission on ExPASy/SIB or at NCBI (USA)		Sequence analysis tools: ProtParam, ProtScale, Compute pI/Mw, PeptideMass, PeptideCutter, Dotlet (Java)
	ScanProsite, MotifScan		Submit a homology modeling request to SWISS-MODEL
	NPSA Sequence analysis tools