Copper-transporting ATPase 2 - Ovis aries (Sheep)

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Alternative products

Sequence annotation (Features)

Sequences

References

Cross-references

Entry information

Relevant documents

Names and origin

Protein attributes

General annotation (Comments)

Ontologies

Alternative products

Sequence annotation (Features)

Sequences

References

Cross-references

Entry information

Relevant documents

Molecule processing

Regions

Sites

Natural variations

Molecule processing

Regions

Sites

Natural variations

Sequence databases

3D structure databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Family and domain databases

Sequence

>sp|Q9XT50|ATP7B_SHEEP Copper-transporting ATPase 2 OS=Ovis aries GN=ATP7B PE=2 SV=1 MERAGDQAPGNPEPSSLATLGDPQVTLLTVHKRWSFKRSPGTGGSSRPVISEEECPPPSE EGEFSQKVLNGSEEISSKQILSKLFQPAMKQSFAFDNNGYEDDLDGVCPSQTAAGTISIV GMTCQSCVKSIEGRVSSLKGIVSIKVSLEQSSAEVRYVPSVVSLMQICHQIEDMGFQASV AEGKATSWASRVSPTSEAVVKLRVEGMTCQSCVSSIEGKIGKLQGVMRVRVSLSNQEAVI TYQPYLIQPQDLRDHITDMGFEAVIKNKVAPVSLGPIDVRRLQSTLSVAPPAPVNQNDNN SETPGGQGVPLHLRVDGMHCKSCVLNIEDNIGQLPGVQSIHVSLESRTARVQYNPSLVSP GALRRAIEALPPGNFKVSFPNGAEGSGPDSRTPPAPSAPCTMMLAIAGMTCKSCVQSIEG LISQRVGVHQISVFLAEGTAVVLYDPSRTHPEELRAAVEDMGFEASILAENCSSNQVGNH SAGSAVGPEAAGAPVPMQGEAPQPGGLHTNHIPHQSPKSLLASTTVAPKKCFLQISGMTC ASCVSNIERNLQKEPGILSVLVALMAGKAEVKYNPEAIQPLEIAKLVQDLGFEAAVMEDY TGSDGDLELMITGMTCASCVHNIESKLRRTEGITYASVALATSKAHVKFDPEIIGPRDIV KLIEEIGFRASLAQRIPNAHHLDHKVEIKQWKNSFLCSLVFGIPVMGLMIYMLIPSHEPQ SSVLDHNVIPGLSILNLIFFILCTFVQFLGGWYFYVQAYKSLRHGMANMDVLIVLATSIA YVYSLVILVVAVAEKAERSPVTFFDTPPMLFVFIALGRWLEHVVKSKTSEALARLMSLQA TEATVVTLGEDNVIIREEQVPMELVQRGDIIKVVPGGKFPVDGKVLEGNTMADESLITGE AMPVTKKPGSMVIAGSMNAHGSVLITATHVGNDTTLAQIVKLVEEAQMSKAPIQQLADRF SGYFVPFIIIISTVTLVVWIVIGFIDFGVVQKYFPAPSKGISQAEVVLRFAFQTSITVLC IACPCSLGLATPTAVMVGTGVAAQNGILIKGGKPLEMAHKIKTVMFDKTGTITHGVPKVS RVLLLVDLATLPLRKVLAVVGTAEASSEHPLGVAVTRYCKEELGTETLGCCMDFQAVPGC GISCKVSSVESILAQGERLQGPPTAHQNRVGSEPSETDAATQTFSVLIGNREWMRRNGLT VTSDVRDAMTDHETKGQTAILVAIDGVLCGMIAVADSVKQEAALAVHTLKSMGVDVVLIT GDNRKTARAIATQVGINKVFAEVLPSHKVAKVQELQNQGKRVAMVGDGVNDSPALAQADV GIAIGTGTDVAIEAADVVLIRNDLLDVVASIHLSRRTVWRIRLNLVLALIYNLIGIPVAA GVFIPIGVVLQPWMGSAAMAASSVSVVLSSLQLKCYRKPDLARYEAQAHGHMKPLSASQV SVRVGMDDRRRDSPRASAWDQVSYVSQVSLSPLKSDKLSRHSGAADDRGDKWSLLLNDRD EEQGI

Options »

Blast tips

You can enter:
• a protein or nucleotide sequence
• a valid UniProt identifier, for example:
P00750 or A4_HUMAN or UPI0000000001
More...

Contribute

Send feedback

Read comments (0) or add your own

Reviewed, UniProtKB/Swiss-Prot Q9XT50 (ATP7B_SHEEP)

Last modified October 13, 2009. Version 77. History...

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

Documents (1) |

Third-party data |

Customize display

text xml rdf/xml gff fasta

Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Alternative products · Sequence annotation (Features) · Sequences · References · Cross-references · Entry information · Relevant documents

Hide | Top

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

Ovis aries (Sheep)

Taxonomic identifier

9940 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Laurasiatheria › Cetartiodactyla › Ruminantia › Pecora › Bovidae › Caprinae › Ovis

Hide | Top

Sequence length	1505 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is not processed.
Protein existence	Evidence at transcript level.

Hide | Top

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.
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	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.
Sequence similarities	Belongs to the cation transport ATPase (P-type) family. Type IB subfamily. Contains 6 HMA domains.

Hide | Top

Keywords
Biological process	Copper transport Ion transport Transport
Cellular component	Golgi apparatus Membrane
Coding sequence diversity	Alternative splicing
Domain	Repeat Transmembrane
Ligand	ATP-binding Copper Magnesium Metal-binding Nucleotide-binding
Molecular function	Hydrolase
PTM	Phosphoprotein
Gene Ontology (GO)
Biological process	ATP biosynthetic process Inferred from electronic annotation. Source: InterPro copper ion transport Inferred from electronic annotation. Source: UniProtKB-KW
Cellular component	Golgi apparatus Inferred from electronic annotation. Source: UniProtKB-KW integral to membrane Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	ATP binding Inferred from electronic annotation. Source: UniProtKB-KW copper ion binding Inferred from electronic annotation. Source: UniProtKB-KW copper-exporting ATPase activity Inferred from electronic annotation. Source: EC magnesium ion binding Inferred from electronic annotation. Source: UniProtKB-KW
Complete GO annotation...

Hide | Top

Hide | Top

Feature key

Position(s)

Length

Description

Graphical view

Feature identifier

Chain

1 – 1505

1505

Copper-transporting ATPase 2

PRO_0000046316

Topological domain

1 – 694

694

Cytoplasmic Potential

Transmembrane

695 – 716

Potential

Topological domain

717 – 737

Extracellular Potential

Transmembrane

738 – 757

Potential

Topological domain

758 – 764

Cytoplasmic Potential

Transmembrane

765 – 785

Potential

Topological domain

786 – 804

Extracellular Potential

Transmembrane

805 – 825

Potential

Topological domain

826 – 959

134

Cytoplasmic Potential

Transmembrane

960 – 982

Potential

Topological domain

983 – 1012

Extracellular Potential

Transmembrane

1013 – 1034

Potential

Topological domain

1035 – 1362

328

Cytoplasmic Potential

Transmembrane

1363 – 1380

Potential

Topological domain

1381 – 1391

Extracellular Potential

Transmembrane

1392 – 1411

Potential

Topological domain

1412 – 1505

Cytoplasmic Potential

Domain

114 – 180

HMA 1

Domain

199 – 265

HMA 2

Domain

310 – 376

HMA 3

Domain

401 – 467

HMA 4

Domain

530 – 596

HMA 5

Domain

606 – 672

HMA 6

Compositional bias

968 – 971

Poly-Ile

Active site

1067

4-aspartylphosphate intermediate By similarity

Metal binding

1307

Magnesium By similarity

Metal binding

1311

Magnesium By similarity

Alternative sequence

1 – 61

Missing in isoform Short.

VSP_000429

Alternative sequence

62 – 79

GEFSQ…ISSKQ → MKPEEERPIIDREKASRR in isoform Short.

VSP_000430

Hide | Top

Sequence		Length	Mass (Da)
Isoform Long [UniParc]. Last modified November 1, 1999. Version 1. Checksum: 63202B55EB2CA8B5 Show »	FASTA	1,505	161,019
10 20 30 40 50 60 MERAGDQAPG NPEPSSLATL GDPQVTLLTV HKRWSFKRSP GTGGSSRPVI SEEECPPPSE 70 80 90 100 110 120 EGEFSQKVLN GSEEISSKQI LSKLFQPAMK QSFAFDNNGY EDDLDGVCPS QTAAGTISIV 130 140 150 160 170 180 GMTCQSCVKS IEGRVSSLKG IVSIKVSLEQ SSAEVRYVPS VVSLMQICHQ IEDMGFQASV 190 200 210 220 230 240 AEGKATSWAS RVSPTSEAVV KLRVEGMTCQ SCVSSIEGKI GKLQGVMRVR VSLSNQEAVI 250 260 270 280 290 300 TYQPYLIQPQ DLRDHITDMG FEAVIKNKVA PVSLGPIDVR RLQSTLSVAP PAPVNQNDNN 310 320 330 340 350 360 SETPGGQGVP LHLRVDGMHC KSCVLNIEDN IGQLPGVQSI HVSLESRTAR VQYNPSLVSP 370 380 390 400 410 420 GALRRAIEAL PPGNFKVSFP NGAEGSGPDS RTPPAPSAPC TMMLAIAGMT CKSCVQSIEG 430 440 450 460 470 480 LISQRVGVHQ ISVFLAEGTA VVLYDPSRTH PEELRAAVED MGFEASILAE NCSSNQVGNH 490 500 510 520 530 540 SAGSAVGPEA AGAPVPMQGE APQPGGLHTN HIPHQSPKSL LASTTVAPKK CFLQISGMTC 550 560 570 580 590 600 ASCVSNIERN LQKEPGILSV LVALMAGKAE VKYNPEAIQP LEIAKLVQDL GFEAAVMEDY 610 620 630 640 650 660 TGSDGDLELM ITGMTCASCV HNIESKLRRT EGITYASVAL ATSKAHVKFD PEIIGPRDIV 670 680 690 700 710 720 KLIEEIGFRA SLAQRIPNAH HLDHKVEIKQ WKNSFLCSLV FGIPVMGLMI YMLIPSHEPQ 730 740 750 760 770 780 SSVLDHNVIP GLSILNLIFF ILCTFVQFLG GWYFYVQAYK SLRHGMANMD VLIVLATSIA 790 800 810 820 830 840 YVYSLVILVV AVAEKAERSP VTFFDTPPML FVFIALGRWL EHVVKSKTSE ALARLMSLQA 850 860 870 880 890 900 TEATVVTLGE DNVIIREEQV PMELVQRGDI IKVVPGGKFP VDGKVLEGNT MADESLITGE 910 920 930 940 950 960 AMPVTKKPGS MVIAGSMNAH GSVLITATHV GNDTTLAQIV KLVEEAQMSK APIQQLADRF 970 980 990 1000 1010 1020 SGYFVPFIII ISTVTLVVWI VIGFIDFGVV QKYFPAPSKG ISQAEVVLRF AFQTSITVLC 1030 1040 1050 1060 1070 1080 IACPCSLGLA TPTAVMVGTG VAAQNGILIK GGKPLEMAHK IKTVMFDKTG TITHGVPKVS 1090 1100 1110 1120 1130 1140 RVLLLVDLAT LPLRKVLAVV GTAEASSEHP LGVAVTRYCK EELGTETLGC CMDFQAVPGC 1150 1160 1170 1180 1190 1200 GISCKVSSVE SILAQGERLQ GPPTAHQNRV GSEPSETDAA TQTFSVLIGN REWMRRNGLT 1210 1220 1230 1240 1250 1260 VTSDVRDAMT DHETKGQTAI LVAIDGVLCG MIAVADSVKQ EAALAVHTLK SMGVDVVLIT 1270 1280 1290 1300 1310 1320 GDNRKTARAI ATQVGINKVF AEVLPSHKVA KVQELQNQGK RVAMVGDGVN DSPALAQADV 1330 1340 1350 1360 1370 1380 GIAIGTGTDV AIEAADVVLI RNDLLDVVAS IHLSRRTVWR IRLNLVLALI YNLIGIPVAA 1390 1400 1410 1420 1430 1440 GVFIPIGVVL QPWMGSAAMA ASSVSVVLSS LQLKCYRKPD LARYEAQAHG HMKPLSASQV 1450 1460 1470 1480 1490 1500 SVRVGMDDRR RDSPRASAWD QVSYVSQVSL SPLKSDKLSR HSGAADDRGD KWSLLLNDRD EEQGI « Hide
Isoform Short. Checksum: FC168B09E685E5D3 Show »	FASTA	1,444	154,808
10 20 30 40 50 60 MKPEEERPII DREKASRRIL SKLFQPAMKQ SFAFDNNGYE DDLDGVCPSQ TAAGTISIVG 70 80 90 100 110 120 MTCQSCVKSI EGRVSSLKGI VSIKVSLEQS SAEVRYVPSV VSLMQICHQI EDMGFQASVA 130 140 150 160 170 180 EGKATSWASR VSPTSEAVVK LRVEGMTCQS CVSSIEGKIG KLQGVMRVRV SLSNQEAVIT 190 200 210 220 230 240 YQPYLIQPQD LRDHITDMGF EAVIKNKVAP VSLGPIDVRR LQSTLSVAPP APVNQNDNNS 250 260 270 280 290 300 ETPGGQGVPL HLRVDGMHCK SCVLNIEDNI GQLPGVQSIH VSLESRTARV QYNPSLVSPG 310 320 330 340 350 360 ALRRAIEALP PGNFKVSFPN GAEGSGPDSR TPPAPSAPCT MMLAIAGMTC KSCVQSIEGL 370 380 390 400 410 420 ISQRVGVHQI SVFLAEGTAV VLYDPSRTHP EELRAAVEDM GFEASILAEN CSSNQVGNHS 430 440 450 460 470 480 AGSAVGPEAA GAPVPMQGEA PQPGGLHTNH IPHQSPKSLL ASTTVAPKKC FLQISGMTCA 490 500 510 520 530 540 SCVSNIERNL QKEPGILSVL VALMAGKAEV KYNPEAIQPL EIAKLVQDLG FEAAVMEDYT 550 560 570 580 590 600 GSDGDLELMI TGMTCASCVH NIESKLRRTE GITYASVALA TSKAHVKFDP EIIGPRDIVK 610 620 630 640 650 660 LIEEIGFRAS LAQRIPNAHH LDHKVEIKQW KNSFLCSLVF GIPVMGLMIY MLIPSHEPQS 670 680 690 700 710 720 SVLDHNVIPG LSILNLIFFI LCTFVQFLGG WYFYVQAYKS LRHGMANMDV LIVLATSIAY 730 740 750 760 770 780 VYSLVILVVA VAEKAERSPV TFFDTPPMLF VFIALGRWLE HVVKSKTSEA LARLMSLQAT 790 800 810 820 830 840 EATVVTLGED NVIIREEQVP MELVQRGDII KVVPGGKFPV DGKVLEGNTM ADESLITGEA 850 860 870 880 890 900 MPVTKKPGSM VIAGSMNAHG SVLITATHVG NDTTLAQIVK LVEEAQMSKA PIQQLADRFS 910 920 930 940 950 960 GYFVPFIIII STVTLVVWIV IGFIDFGVVQ KYFPAPSKGI SQAEVVLRFA FQTSITVLCI 970 980 990 1000 1010 1020 ACPCSLGLAT PTAVMVGTGV AAQNGILIKG GKPLEMAHKI KTVMFDKTGT ITHGVPKVSR 1030 1040 1050 1060 1070 1080 VLLLVDLATL PLRKVLAVVG TAEASSEHPL GVAVTRYCKE ELGTETLGCC MDFQAVPGCG 1090 1100 1110 1120 1130 1140 ISCKVSSVES ILAQGERLQG PPTAHQNRVG SEPSETDAAT QTFSVLIGNR EWMRRNGLTV 1150 1160 1170 1180 1190 1200 TSDVRDAMTD HETKGQTAIL VAIDGVLCGM IAVADSVKQE AALAVHTLKS MGVDVVLITG 1210 1220 1230 1240 1250 1260 DNRKTARAIA TQVGINKVFA EVLPSHKVAK VQELQNQGKR VAMVGDGVND SPALAQADVG 1270 1280 1290 1300 1310 1320 IAIGTGTDVA IEAADVVLIR NDLLDVVASI HLSRRTVWRI RLNLVLALIY NLIGIPVAAG 1330 1340 1350 1360 1370 1380 VFIPIGVVLQ PWMGSAAMAA SSVSVVLSSL QLKCYRKPDL ARYEAQAHGH MKPLSASQVS 1390 1400 1410 1420 1430 1440 VRVGMDDRRR DSPRASAWDQ VSYVSQVSLS PLKSDKLSRH SGAADDRGDK WSLLLNDRDE EQGI « Hide

Hide | Top

[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: 10760584] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS LONG AND SHORT).

Hide | Top

Sequence databases
	AF118225 mRNA. Translation: AAD39371.1. AF032881 mRNA. Translation: AAB94620.1.
RefSeq	NP_001009732.1.
UniGene	Oar.747
3D structure databases
HSSP	HSSP built from PDB template 1AW0 based on UniProtKB Q04656.
SMR	Q9XT50. Positions 526-674, 1074-1236.
ModBase	Search...
Genome annotation databases
GeneID	443046.
Organism-specific databases
CTD	443046.
Phylogenomic databases
HOVERGEN	Q9XT50.
Enzyme and pathway databases
BRENDA	3.6.3.4. 271.
Family and domain databases
InterPro	IPR001877. ATPase1_Cu-transp. IPR008250. ATPase_P-typ_ATPase-assoc-reg. IPR006403. ATPase_P-typ_cat/Cu-transptr. IPR006416. ATPase_P-typ_heavy-metal. IPR001757. ATPase_P-typ_ion-transptr. IPR018303. ATPase_P-typ_P_site. IPR006122. Cu_ion_bd. IPR005834. Dehalogen-like_hydro. IPR017969. Heavy-metal-associated_CS. IPR006121. HeavyMe_transpt. [Graphical view]
PANTHER	PTHR11939. ATPase_P. 1 hit.
Pfam	PF00122. E1-E2_ATPase. 1 hit. PF00403. HMA. 6 hits. PF00702. Hydrolase. 1 hit. [Graphical view]
PRINTS	PR00119. CATATPASE. PR00942. CUATPASEI.
TIGRFAMs	TIGR01511. ATPase-IB1_Cu. 1 hit. TIGR01525. ATPase-IB_hvy. 1 hit. TIGR01494. ATPase_P-type. 1 hit. TIGR00003. Cu_ion_bd. 2 hits.
PROSITE	PS00154. ATPASE_E1_E2. 1 hit. PS01047. HMA_1. 6 hits. PS50846. HMA_2. 6 hits. [Graphical view]
ProtoNet	Search...

Hide | Top

Entry name

ATP7B_SHEEP

Accession

Primary (citable) accession number: Q9XT50
Secondary accession number(s): O46518

Entry history

Integrated into UniProtKB/Swiss-Prot:	December 8, 2000
Last sequence update:	November 1, 1999
Last modified:	October 13, 2009
This is version 77 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)

Hide | Top

SIMILARITY comments

Index of protein domains and families

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform Long (identifier: Q9XT50-1)

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.

Isoform Short (identifier: Q9XT50-2)

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