Names & Taxonomy

Uniprot ID:
Q04656
Entry Name:
ATP7A_HUMAN
Status:
reviewed
Protein Names:
Copper-transporting ATPase 1 (EC 3.6.3.54) (Copper pump 1) (Menkes disease-associated protein)
Gene Names:
ATP7A MC1 MNK
Gene Names Primary:
ATP7A
Organism:
Homo sapiens (Human)

Structure

Length:
1500
Sequence:
MDPSMGVNSVTISVEGMTCNSCVWTIEQQIGKVNGVHHIKVSLEEKNATIIYDPKLQTPKTLQEAIDDMGFDAVIHNPDPLPVLTDTLFLTVTASLTLPWDHIQSTLLKTKGVTDIKIYPQKRTVAVTIIPSIVNANQIKELVPELSLDTGTLEKKSGACEDHSMAQAGEVVLKMKVEGMTCHSCTSTIEGKIGKLQGVQRIKVSLDNQEATIVYQPHLISVEEMKKQIEAMGFPAFVKKQPKYLKLGAIDVERLKNTPVKSSEGSQQRSPSYTNDSTATFIIDGMHCKSCVSNIESTLSALQYVSSIVVSLENRSAIVKYNASSVTPESLRKAIEAVSPGLYRVSITSEVESTSNSPSSSSLQKIPLNVVSQPLTQETVINIDGMTCNSCVQSIEGVISKKPGVKSIRVSLANSNGTVEYDPLLTSPETLRGAIEDMGFDATLSDTNEPLVVIAQPSSEMPLLTSTNEFYTKGMTPVQDKEEGKNSSKCYIQVTGMTCASCVANIERNLRREEGIYSILVALMAGKAEVRYNPAVIQPPMIAEFIRELGFGATVIENADEGDGVLELVVRGMTCASCVHKIESSLTKHRGILYCSVALATNKAHIKYDPEIIGPRDIIHTIESLGFEASLVKKDRSASHLDHKREIRQWRRSFLVSLFFCIPVMGLMIYMMVMDHHFATLHHNQNMSKEEMINLHSSMFLERQILPGLSVMNLLSFLLCVPVQFFGGWYFYIQAYKALKHKTANMDVLIVLATTIAFAYSLIILLVAMYERAKVNPITFFDTPPMLFVFIALGRWLEHIAKGKTSEALAKLISLQATEATIVTLDSDNILLSEEQVDVELVQRGDIIKVVPGGKFPVDGRVIEGHSMVDESLITGEAMPVAKKPGSTVIAGSINQNGSLLICATHVGADTTLSQIVKLVEEAQTSKAPIQQFADKLSGYFVPFIVFVSIATLLVWIVIGFLNFEIVETYFPGYNRSISRTETIIRFAFQASITVLCIACPCSLGLATPTAVMVGTGVGAQNGILIKGGEPLEMAHKVKVVVFDKTGTITHGTPVVNQVKVLTESNRISHHKILAIVGTAESNSEHPLGTAITKYCKQELDTETLGTCIDFQVVPGCGISCKVTNIEGLLHKNNWNIEDNNIKNASLVQIDASNEQSSTSSSMIIDAQISNALNAQQYKVLIGNREWMIRNGLVINNDVNDFMTEHERKGRTAVLVAVDDELCGLIAIADTVKPEAELAIHILKSMGLEVVLMTGDNSKTARSIASQVGITKVFAEVLPSHKVAKVKQLQEEGKRVAMVGDGINDSPALAMANVGIAIGTGTDVAIEAADVVLIRNDLLDVVASIDLSRETVKRIRINFVFALIYNLVGIPIAAGVFMPIGLVLQPWMGSAAMAASSVSVVLSSLFLKLYRKPTYESYELPARSQIGQKSPSEISVHVGIDDTSRNSPKLGLLDRIVNYSRASINSLLSDKRSLNSVVTSEPDKHSLLVGDFREDDDTAL
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Golgi apparatus, trans-Golgi network membrane

Function

Function:
May supply copper to copper-requiring proteins within the secretory pathway, when localized in the trans-Golgi network. Under conditions of elevated extracellular copper, it relocalized to the plasma membrane where it functions in the efflux of copper from cells.
Catalytic Activity:
ATP + H(2)O + Cu(+)(Side 1) = ADP + phosphate + Cu(+)(Side 2).
Active Site:
ACT_SITE 1044 1044 4-aspartylphosphate intermediate.
Cross Reference Drug Bank:
DB00958 DB00515 DB00526
Gene Ontology Go:
basolateral plasma membrane
brush border membrane
cytosol
endoplasmic reticulum
Golgi apparatus
integral component of plasma membrane
late endosome
membrane
neuron projection
neuronal cell body
perinuclear region of cytoplasm
plasma membrane
secretory granule
trans-Golgi network
trans-Golgi network transport vesicle
ATP binding
copper ion binding
copper ion transmembrane transporter activity
copper-dependent protein binding
copper-exporting ATPase activity
superoxide dismutase copper chaperone activity
blood vessel development
blood vessel remodeling
cartilage development
catecholamine metabolic process
cellular copper ion homeostasis
central nervous system neuron development
cerebellar Purkinje cell differentiation
collagen fibril organization
copper ion export
copper ion import
copper ion transport
detoxification of copper ion
dopamine metabolic process
elastic fiber assembly
elastin biosynthetic process
epinephrine metabolic process
extracellular matrix organization
hair follicle morphogenesis
in utero embryonic development
ion transmembrane transport
lactation
locomotory behavior
lung alveolus development
mitochondrion organization
negative regulation of metalloenzyme activity
neuron projection morphogenesis
norepinephrine metabolic process
peptidyl-lysine modification
pigmentation
plasma membrane copper ion transport
positive regulation of catalytic activity
positive regulation of metalloenzyme activity
positive regulation of oxidoreductase activity
pyramidal neuron development
regulation of oxidative phosphorylation
removal of superoxide radicals
response to iron(III) ion
response to reactive oxygen species
response to zinc ion
serotonin metabolic process
skin development
T-helper cell differentiation
transmembrane transport
tryptophan metabolic process
Gene Ontology Biological Process:
blood vessel development
blood vessel remodeling
cartilage development
catecholamine metabolic process
cellular copper ion homeostasis
central nervous system neuron development
cerebellar Purkinje cell differentiation
collagen fibril organization
copper ion export
copper ion import
copper ion transport
detoxification of copper ion
dopamine metabolic process
elastic fiber assembly
elastin biosynthetic process
epinephrine metabolic process
extracellular matrix organization
hair follicle morphogenesis
in utero embryonic development
ion transmembrane transport
lactation
locomotory behavior
lung alveolus development
mitochondrion organization
negative regulation of metalloenzyme activity
neuron projection morphogenesis
norepinephrine metabolic process
peptidyl-lysine modification
pigmentation
plasma membrane copper ion transport
positive regulation of catalytic activity
positive regulation of metalloenzyme activity
positive regulation of oxidoreductase activity
pyramidal neuron development
regulation of oxidative phosphorylation
removal of superoxide radicals
response to iron(III) ion
response to reactive oxygen species
response to zinc ion
serotonin metabolic process
skin development
T-helper cell differentiation
transmembrane transport
tryptophan metabolic process
Gene Ontology Molecular Function:
ATP binding
copper-dependent protein binding
copper-exporting ATPase activity
copper ion binding
copper ion transmembrane transporter activity
superoxide dismutase copper chaperone activity
Gene Ontology Cellular Component:
basolateral plasma membrane
brush border membrane
cytosol
endoplasmic reticulum
Golgi apparatus
integral component of plasma membrane
late endosome
membrane
neuronal cell body
neuron projection
perinuclear region of cytoplasm
plasma membrane
secretory granule
trans-Golgi network
trans-Golgi network transport vesicle
Keywords:
3D-structure
ATP-binding
Alternative splicing
Cell membrane
Complete proteome
Copper
Copper transport
Cytoplasm
Disease mutation
Endoplasmic reticulum
Glycoprotein
Golgi apparatus
Hydrolase
Ion transport
Magnesium
Membrane
Metal-binding
Neurodegeneration
Nucleotide-binding
Phosphoprotein
Polymorphism
Reference proteome
Repeat
Transmembrane
Transmembrane helix
Transport
Interacts With:
Q5EBL8

Publication

PubMed ID:
8490659 7607665 9693104 10079814 15772651 7490081 8490646 8490647 11214319 9467005 10970802 9147644 10484781 16051599 19690332 20068231 24275569 9437429 10079817 7977350 8981948 9246006 10401004 10319589 11431706 11241493 11350187 15981243 17108763 20170900 22992316