Names & Taxonomy

Uniprot ID:
P35670
Entry Name:
ATP7B_HUMAN
Status:
reviewed
Protein Names:
Copper-transporting ATPase 2 (EC 3.6.3.54) (Copper pump 2) (Wilson disease-associated protein) [Cleaved into: WND/140 kDa]
Gene Names:
ATP7B PWD WC1 WND
Gene Names Primary:
ATP7B
Organism:
Homo sapiens (Human)

Structure

Length:
1465
Sequence:
MPEQERQITAREGASRKILSKLSLPTRAWEPAMKKSFAFDNVGYEGGLDGLGPSSQVATSTVRILGMTCQSCVKSIEDRISNLKGIISMKVSLEQGSATVKYVPSVVCLQQVCHQIGDMGFEASIAEGKAASWPSRSLPAQEAVVKLRVEGMTCQSCVSSIEGKVRKLQGVVRVKVSLSNQEAVITYQPYLIQPEDLRDHVNDMGFEAAIKSKVAPLSLGPIDIERLQSTNPKRPLSSANQNFNNSETLGHQGSHVVTLQLRIDGMHCKSCVLNIEENIGQLLGVQSIQVSLENKTAQVKYDPSCTSPVALQRAIEALPPGNFKVSLPDGAEGSGTDHRSSSSHSPGSPPRNQVQGTCSTTLIAIAGMTCASCVHSIEGMISQLEGVQQISVSLAEGTATVLYNPSVISPEELRAAIEDMGFEASVVSESCSTNPLGNHSAGNSMVQTTDGTPTSVQEVAPHTGRLPANHAPDILAKSPQSTRAVAPQKCFLQIKGMTCASCVSNIERNLQKEAGVLSVLVALMAGKAEIKYDPEVIQPLEIAQFIQDLGFEAAVMEDYAGSDGNIELTITGMTCASCVHNIESKLTRTNGITYASVALATSKALVKFDPEIIGPRDIIKIIEEIGFHASLAQRNPNAHHLDHKMEIKQWKKSFLCSLVFGIPVMALMIYMLIPSNEPHQSMVLDHNIIPGLSILNLIFFILCTFVQLLGGWYFYVQAYKSLRHRSANMDVLIVLATSIAYVYSLVILVVAVAEKAERSPVTFFDTPPMLFVFIALGRWLEHLAKSKTSEALAKLMSLQATEATVVTLGEDNLIIREEQVPMELVQRGDIVKVVPGGKFPVDGKVLEGNTMADESLITGEAMPVTKKPGSTVIAGSINAHGSVLIKATHVGNDTTLAQIVKLVEEAQMSKAPIQQLADRFSGYFVPFIIIMSTLTLVVWIVIGFIDFGVVQRYFPNPNKHISQTEVIIRFAFQTSITVLCIACPCSLGLATPTAVMVGTGVAAQNGILIKGGKPLEMAHKIKTVMFDKTGTITHGVPRVMRVLLLGDVATLPLRKVLAVVGTAEASSEHPLGVAVTKYCKEELGTETLGYCTDFQAVPGCGIGCKVSNVEGILAHSERPLSAPASHLNEAGSLPAEKDAVPQTFSVLIGNREWLRRNGLTISSDVSDAMTDHEMKGQTAILVAIDGVLCGMIAIADAVKQEAALAVHTLQSMGVDVVLITGDNRKTARAIATQVGINKVFAEVLPSHKVAKVQELQNKGKKVAMVGDGVNDSPALAQADMGVAIGTGTDVAIEAADVVLIRNDLLDVVASIHLSKRTVRRIRINLVLALIYNLVGIPIAAGVFMPIGIVLQPWMGSAAMAASSVSVVLSSLQLKCYKKPDLERYEAQAHGHMKPLTASQVSVHIGMDDRWRDSPRATPWDQVSYVSQVSLSSLTSDKPSRHSAAADDDGDKWSLLLNGRDEEQYI
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Golgi apparatus, trans-Golgi network membrane

Function

Function:
Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
Catalytic Activity:
ATP + H(2)O + Cu(+)(Side 1) = ADP + phosphate + Cu(+)(Side 2).
Active Site:
ACT_SITE 1027 1027 4-aspartylphosphate intermediate.
Cross Reference Drug Bank:
DB00958 DB00515 DB00526
Gene Ontology Go:
bicellular tight junction
Golgi membrane
integral component of plasma membrane
late endosome
membrane
mitochondrion
trans-Golgi network membrane
ATP binding
copper ion binding
copper-exporting ATPase activity
copper-transporting ATPase activity
zinc ion binding
cellular copper ion homeostasis
cellular zinc ion homeostasis
circadian rhythm
copper ion import
copper ion transport
intracellular copper ion transport
ion transmembrane transport
lactation
response to cAMP
response to copper ion
response to zinc ion
sequestering of calcium ion
transmembrane transport
Gene Ontology Biological Process:
cellular copper ion homeostasis
cellular zinc ion homeostasis
circadian rhythm
copper ion import
copper ion transport
intracellular copper ion transport
ion transmembrane transport
lactation
response to cAMP
response to copper ion
response to zinc ion
sequestering of calcium ion
transmembrane transport
Gene Ontology Molecular Function:
ATP binding
copper-exporting ATPase activity
copper ion binding
copper-transporting ATPase activity
zinc ion binding
Gene Ontology Cellular Component:
bicellular tight junction
Golgi membrane
integral component of plasma membrane
late endosome
membrane
mitochondrion
trans-Golgi network membrane
Keywords:
3D-structure
ATP-binding
Alternative splicing
Complete proteome
Copper
Copper transport
Cytoplasm
Disease mutation
Golgi apparatus
Hydrolase
Ion transport
Magnesium
Membrane
Metal-binding
Mitochondrion
Nucleotide-binding
Phosphoprotein
Polymorphism
Reference proteome
Repeat
Transmembrane
Transmembrane helix
Transport

Publication

PubMed ID:
7833924 15057823 10334941 8298639 8298641 8250934 7626145 9307043 9837819 9600907 12968035 16554302 20032459 22240481 24706876 18558714 8533760 8938442 8931691 8782057 9311736 9772425 9222767 8980283 9887381 9482578 9554743 9452121 9671269 9829905 10194254 10447265 10502776 10502777 10051024 10544227 10453196 11216666 11093740 10790207 10721669 11043508 11180609 11690702 11243728 11954751 12544487 12325021 12376745 14986826 14639035 15024742 15557537 14966923 15845031 15811015 15952988 16207219 16283883 16088907 15967699 17718866 18373411 18203200 21682854 23333878 24303094