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Protein

Copper-transporting ATPase 2

Gene

ATP7B

Organism
Ovis aries (Sheep)
Status
Reviewed-Annotation score: Annotation score: 4 out of 5-Experimental evidence at transcript 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).By similarity

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi124 – 1241Copper 1PROSITE-ProRule annotation
Metal bindingi127 – 1271Copper 1PROSITE-ProRule annotation
Metal bindingi209 – 2091Copper 2PROSITE-ProRule annotation
Metal bindingi212 – 2121Copper 2PROSITE-ProRule annotation
Metal bindingi320 – 3201Copper 3PROSITE-ProRule annotation
Metal bindingi323 – 3231Copper 3PROSITE-ProRule annotation
Metal bindingi411 – 4111Copper 4PROSITE-ProRule annotation
Metal bindingi414 – 4141Copper 4PROSITE-ProRule annotation
Metal bindingi540 – 5401Copper 5PROSITE-ProRule annotation
Metal bindingi543 – 5431Copper 5PROSITE-ProRule annotation
Metal bindingi616 – 6161Copper 6PROSITE-ProRule annotation
Metal bindingi619 – 6191Copper 6PROSITE-ProRule annotation
Active sitei1067 – 106714-aspartylphosphate intermediateBy similarity
Metal bindingi1307 – 13071MagnesiumPROSITE-ProRule annotation
Metal bindingi1311 – 13111MagnesiumPROSITE-ProRule annotation

GO - Molecular functioni

GO - Biological processi

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

Names & Taxonomyi

Protein namesi
Recommended name:
Copper-transporting ATPase 2 (EC:3.6.3.54By similarity)
Alternative name(s):
Copper pump 2
Wilson disease-associated protein homolog
Gene namesi
Name:ATP7B
Synonyms:WND
OrganismiOvis aries (Sheep)
Taxonomic identifieri9940 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaRuminantiaPecoraBovidaeCaprinaeOvis
Proteomesi
  • UP000002356 Componenti: Unplaced

Subcellular locationi

  • Golgi apparatustrans-Golgi network membrane By similarity; Multi-pass membrane protein Sequence analysisBy 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

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 694694CytoplasmicSequence analysisAdd
BLAST
Transmembranei695 – 71622HelicalSequence analysisAdd
BLAST
Topological domaini717 – 73721ExtracellularSequence analysisAdd
BLAST
Transmembranei738 – 75720HelicalSequence analysisAdd
BLAST
Topological domaini758 – 7647CytoplasmicSequence analysis
Transmembranei765 – 78521HelicalSequence analysisAdd
BLAST
Topological domaini786 – 80419ExtracellularSequence analysisAdd
BLAST
Transmembranei805 – 82521HelicalSequence analysisAdd
BLAST
Topological domaini826 – 959134CytoplasmicSequence analysisAdd
BLAST
Transmembranei960 – 98223HelicalSequence analysisAdd
BLAST
Topological domaini983 – 101230ExtracellularSequence analysisAdd
BLAST
Transmembranei1013 – 103422HelicalSequence analysisAdd
BLAST
Topological domaini1035 – 1362328CytoplasmicSequence analysisAdd
BLAST
Transmembranei1363 – 138018HelicalSequence analysisAdd
BLAST
Topological domaini1381 – 139111ExtracellularSequence analysisAdd
BLAST
Transmembranei1392 – 141120HelicalSequence analysisAdd
BLAST
Topological domaini1412 – 150594CytoplasmicSequence analysisAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Golgi apparatus, Membrane

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 15051505Copper-transporting ATPase 2PRO_0000046316Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei519 – 5191PhosphoserineBy similarity
Modified residuei1438 – 14381PhosphoserineBy similarity

Keywords - PTMi

Phosphoprotein

Expressioni

Tissue specificityi

The short isoform is expressed primarily in the liver with lower levels present in the intestine, hypothalamus and ovary. The long isoform is expressed in the liver.

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

Structurei

3D structure databases

ProteinModelPortaliQ9XT50.
SMRiQ9XT50. Positions 197-266, 312-381, 526-674, 1074-1236.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini114 – 18067HMA 1PROSITE-ProRule annotationAdd
BLAST
Domaini199 – 26567HMA 2PROSITE-ProRule annotationAdd
BLAST
Domaini310 – 37667HMA 3PROSITE-ProRule annotationAdd
BLAST
Domaini401 – 46767HMA 4PROSITE-ProRule annotationAdd
BLAST
Domaini530 – 59667HMA 5PROSITE-ProRule annotationAdd
BLAST
Domaini606 – 67267HMA 6PROSITE-ProRule annotationAdd
BLAST

Compositional bias

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Compositional biasi968 – 9714Poly-Ile

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

HOVERGENiHBG050616.
KOiK17686.

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.
IPR023214. HAD-like_dom.
IPR017969. Heavy-metal-associated_CS.
IPR006122. HMA_Cu_ion-bd.
IPR006121. HMA_dom.
IPR027256. P-typ_ATPase_IB.
IPR001757. P_typ_ATPase.
[Graphical view]
PfamiPF00122. E1-E2_ATPase. 1 hit.
PF00403. HMA. 6 hits.
[Graphical view]
SUPFAMiSSF55008. SSF55008. 6 hits.
SSF56784. SSF56784. 2 hits.
TIGRFAMsiTIGR01525. ATPase-IB_hvy. 1 hit.
TIGR01494. ATPase_P-type. 2 hits.
TIGR00003. TIGR00003. 6 hits.
PROSITEiPS00154. ATPASE_E1_E2. 1 hit.
PS01047. HMA_1. 6 hits.
PS50846. HMA_2. 6 hits.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform Long (identifier: Q9XT50-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MERAGDQAPG NPEPSSLATL GDPQVTLLTV HKRWSFKRSP GTGGSSRPVI
60 70 80 90 100
SEEECPPPSE EGEFSQKVLN GSEEISSKQI LSKLFQPAMK QSFAFDNNGY
110 120 130 140 150
EDDLDGVCPS QTAAGTISIV GMTCQSCVKS IEGRVSSLKG IVSIKVSLEQ
160 170 180 190 200
SSAEVRYVPS VVSLMQICHQ IEDMGFQASV AEGKATSWAS RVSPTSEAVV
210 220 230 240 250
KLRVEGMTCQ SCVSSIEGKI GKLQGVMRVR VSLSNQEAVI TYQPYLIQPQ
260 270 280 290 300
DLRDHITDMG FEAVIKNKVA PVSLGPIDVR RLQSTLSVAP PAPVNQNDNN
310 320 330 340 350
SETPGGQGVP LHLRVDGMHC KSCVLNIEDN IGQLPGVQSI HVSLESRTAR
360 370 380 390 400
VQYNPSLVSP GALRRAIEAL PPGNFKVSFP NGAEGSGPDS RTPPAPSAPC
410 420 430 440 450
TMMLAIAGMT CKSCVQSIEG LISQRVGVHQ ISVFLAEGTA VVLYDPSRTH
460 470 480 490 500
PEELRAAVED MGFEASILAE NCSSNQVGNH SAGSAVGPEA AGAPVPMQGE
510 520 530 540 550
APQPGGLHTN HIPHQSPKSL LASTTVAPKK CFLQISGMTC ASCVSNIERN
560 570 580 590 600
LQKEPGILSV LVALMAGKAE VKYNPEAIQP LEIAKLVQDL GFEAAVMEDY
610 620 630 640 650
TGSDGDLELM ITGMTCASCV HNIESKLRRT EGITYASVAL ATSKAHVKFD
660 670 680 690 700
PEIIGPRDIV KLIEEIGFRA SLAQRIPNAH HLDHKVEIKQ WKNSFLCSLV
710 720 730 740 750
FGIPVMGLMI YMLIPSHEPQ SSVLDHNVIP GLSILNLIFF ILCTFVQFLG
760 770 780 790 800
GWYFYVQAYK SLRHGMANMD VLIVLATSIA YVYSLVILVV AVAEKAERSP
810 820 830 840 850
VTFFDTPPML FVFIALGRWL EHVVKSKTSE ALARLMSLQA TEATVVTLGE
860 870 880 890 900
DNVIIREEQV PMELVQRGDI IKVVPGGKFP VDGKVLEGNT MADESLITGE
910 920 930 940 950
AMPVTKKPGS MVIAGSMNAH GSVLITATHV GNDTTLAQIV KLVEEAQMSK
960 970 980 990 1000
APIQQLADRF SGYFVPFIII ISTVTLVVWI VIGFIDFGVV QKYFPAPSKG
1010 1020 1030 1040 1050
ISQAEVVLRF AFQTSITVLC IACPCSLGLA TPTAVMVGTG VAAQNGILIK
1060 1070 1080 1090 1100
GGKPLEMAHK IKTVMFDKTG TITHGVPKVS RVLLLVDLAT LPLRKVLAVV
1110 1120 1130 1140 1150
GTAEASSEHP LGVAVTRYCK EELGTETLGC CMDFQAVPGC GISCKVSSVE
1160 1170 1180 1190 1200
SILAQGERLQ GPPTAHQNRV GSEPSETDAA TQTFSVLIGN REWMRRNGLT
1210 1220 1230 1240 1250
VTSDVRDAMT DHETKGQTAI LVAIDGVLCG MIAVADSVKQ EAALAVHTLK
1260 1270 1280 1290 1300
SMGVDVVLIT GDNRKTARAI ATQVGINKVF AEVLPSHKVA KVQELQNQGK
1310 1320 1330 1340 1350
RVAMVGDGVN DSPALAQADV GIAIGTGTDV AIEAADVVLI RNDLLDVVAS
1360 1370 1380 1390 1400
IHLSRRTVWR IRLNLVLALI YNLIGIPVAA GVFIPIGVVL QPWMGSAAMA
1410 1420 1430 1440 1450
ASSVSVVLSS LQLKCYRKPD LARYEAQAHG HMKPLSASQV SVRVGMDDRR
1460 1470 1480 1490 1500
RDSPRASAWD QVSYVSQVSL SPLKSDKLSR HSGAADDRGD KWSLLLNDRD

EEQGI
Length:1,505
Mass (Da):161,019
Last modified:November 1, 1999 - v1
Checksum:i63202B55EB2CA8B5
GO
Isoform Short (identifier: Q9XT50-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-61: Missing.
     62-79: GEFSQKVLNGSEEISSKQ → MKPEEERPIIDREKASRR

Show »
Length:1,444
Mass (Da):154,808
Checksum:iFC168B09E685E5D3
GO

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 6161Missing in isoform Short. 1 PublicationVSP_000429Add
BLAST
Alternative sequencei62 – 7918GEFSQ…ISSKQ → MKPEEERPIIDREKASRR in isoform Short. 1 PublicationVSP_000430Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF118225 mRNA. Translation: AAD39371.1.
AF032881 mRNA. Translation: AAB94620.1.
RefSeqiNP_001009732.1. NM_001009732.1. [Q9XT50-1]
UniGeneiOar.747.

Genome annotation databases

GeneIDi443046.
KEGGioas:443046.

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF118225 mRNA. Translation: AAD39371.1.
AF032881 mRNA. Translation: AAB94620.1.
RefSeqiNP_001009732.1. NM_001009732.1. [Q9XT50-1]
UniGeneiOar.747.

3D structure databases

ProteinModelPortaliQ9XT50.
SMRiQ9XT50. Positions 197-266, 312-381, 526-674, 1074-1236.
ModBaseiSearch...
MobiDBiSearch...

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

GeneIDi443046.
KEGGioas:443046.

Organism-specific databases

CTDi540.

Phylogenomic databases

HOVERGENiHBG050616.
KOiK17686.

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.
IPR023214. HAD-like_dom.
IPR017969. Heavy-metal-associated_CS.
IPR006122. HMA_Cu_ion-bd.
IPR006121. HMA_dom.
IPR027256. P-typ_ATPase_IB.
IPR001757. P_typ_ATPase.
[Graphical view]
PfamiPF00122. E1-E2_ATPase. 1 hit.
PF00403. HMA. 6 hits.
[Graphical view]
SUPFAMiSSF55008. SSF55008. 6 hits.
SSF56784. SSF56784. 2 hits.
TIGRFAMsiTIGR01525. ATPase-IB_hvy. 1 hit.
TIGR01494. ATPase_P-type. 2 hits.
TIGR00003. TIGR00003. 6 hits.
PROSITEiPS00154. ATPASE_E1_E2. 1 hit.
PS01047. HMA_1. 6 hits.
PS50846. HMA_2. 6 hits.
[Graphical view]
ProtoNetiSearch...

Publicationsi

  1. "Cloning, mapping and expression analysis of the sheep wilson disease gene homologue."
    Lockhart P.J., Wilcox S.A., Dahl H.-H.M., Mercer J.F.B.
    Biochim. Biophys. Acta 1491:229-239(2000) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS LONG AND SHORT).

Entry informationi

Entry nameiATP7B_SHEEP
AccessioniPrimary (citable) accession number: Q9XT50
Secondary accession number(s): O46518
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 8, 2000
Last sequence update: November 1, 1999
Last modified: July 6, 2016
This is version 116 of the entry and version 1 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. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.