ATP7A
UniProt ID: Q04656
Entrez ID: 538
Protein name: Copper-transporting ATPase 1 (EC 7.2.2.8) (Copper pump 1) (Menkes disease-associated protein)
External links: UniprotKB HGNC RefSeq COSMIC
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FUNCTION: ATP-driven copper (Cu(+)) ion pump that plays an important role in intracellular copper ion homeostasis (PubMed:10419525, PubMed:11092760, PubMed:28389643). Within a catalytic cycle, acquires Cu(+) ion from donor protein on the cytoplasmic side of the membrane and delivers it to acceptor p...
FUNCTION: ATP-driven copper (Cu(+)) ion pump that plays an important role in intracellular copper ion homeostasis (PubMed:10419525, PubMed:11092760, PubMed:28389643). Within a catalytic cycle, acquires Cu(+) ion from donor protein on the cytoplasmic side of the membrane and delivers it to acceptor protein on the lumenal side. The transfer of Cu(+) ion across the membrane is coupled to ATP hydrolysis and is associated with a transient phosphorylation that shifts the pump conformation from inward-facing to outward-facing state (PubMed:10419525, PubMed:19453293, PubMed:19917612, PubMed:31283225, PubMed:28389643). Under physiological conditions, at low cytosolic copper concentration, it is localized at the trans-Golgi network (TGN) where it transfers Cu(+) ions to cuproenzymes of the secretory pathway (PubMed:28389643, PubMed:11092760). Upon elevated cytosolic copper concentrations, it relocalizes to the plasma membrane where it is responsible for the export of excess Cu(+) ions (PubMed:10419525, PubMed:28389643). May play a dual role in neuron function and survival by regulating cooper efflux and neuronal transmission at the synapse as well as by supplying Cu(+) ions to enzymes such as PAM, TYR and SOD3 (PubMed:28389643) (By similarity). In the melanosomes of pigmented cells, provides copper cofactor to TYR to form an active TYR holoenzyme for melanin biosynthesis (By similarity). {ECO:0000250|UniProtKB:Q64430, ECO:0000269|PubMed:10419525, ECO:0000269|PubMed:11092760, ECO:0000269|PubMed:19453293, ECO:0000269|PubMed:19917612, ECO:0000269|PubMed:28389643, ECO:0000269|PubMed:31283225}.
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GO - Biological processes (BP):
blood vessel development [GO:0001568]; blood vessel remodeling [GO:0001974]; cartilage development [GO:0051216]; catecholamine metabolic process [GO:0006584]; cellular copper ion homeostasis [GO:0006878]; cellular response to amino acid stimulus [GO:0071230]; cellular response to antibiotic [GO:0071...
blood vessel development [GO:0001568]; blood vessel remodeling [GO:0001974]; cartilage development [GO:0051216]; catecholamine metabolic process [GO:0006584]; cellular copper ion homeostasis [GO:0006878]; cellular response to amino acid stimulus [GO:0071230]; cellular response to antibiotic [GO:0071236]; cellular response to cadmium ion [GO:0071276]; cellular response to cobalt ion [GO:0071279]; cellular response to copper ion [GO:0071280]; cellular response to hypoxia [GO:0071456]; cellular response to iron ion [GO:0071281]; cellular response to lead ion [GO:0071284]; cellular response to platelet-derived growth factor stimulus [GO:0036120]; central nervous system neuron development [GO:0021954]; cerebellar Purkinje cell differentiation [GO:0021702]; collagen fibril organization [GO:0030199]; copper ion export [GO:0060003]; copper ion homeostasis [GO:0055070]; copper ion import [GO:0015677]; copper ion transport [GO:0006825]; detoxification of copper ion [GO:0010273]; dopamine metabolic process [GO:0042417]; elastic fiber assembly [GO:0048251]; elastin biosynthetic process [GO:0051542]; epinephrine metabolic process [GO:0042414]; extracellular matrix organization [GO:0030198]; female pregnancy [GO:0007565]; hair follicle morphogenesis [GO:0031069]; in utero embryonic development [GO:0001701]; lactation [GO:0007595]; liver development [GO:0001889]; locomotory behavior [GO:0007626]; lung alveolus development [GO:0048286]; mitochondrion organization [GO:0007005]; negative regulation of iron ion transmembrane transport [GO:0034760]; neuron projection morphogenesis [GO:0048812]; norepinephrine metabolic process [GO:0042415]; peptidyl-lysine modification [GO:0018205]; pigmentation [GO:0043473]; positive regulation of catalytic activity [GO:0043085]; positive regulation of cell size [GO:0045793]; positive regulation of epithelial cell proliferation [GO:0050679]; positive regulation of lamellipodium assembly [GO:0010592]; positive regulation of melanin biosynthetic process [GO:0048023]; positive regulation of response to wounding [GO:1903036]; positive regulation of tyrosinase activity [GO:0032773]; positive regulation of vascular associated smooth muscle cell migration [GO:1904754]; pyramidal neuron development [GO:0021860]; regulation of cytochrome-c oxidase activity [GO:1904959]; regulation of gene expression [GO:0010468]; regulation of oxidative phosphorylation [GO:0002082]; removal of superoxide radicals [GO:0019430]; response to iron(III) ion [GO:0010041]; response to manganese ion [GO:0010042]; response to zinc ion [GO:0010043]; serotonin metabolic process [GO:0042428]; skin development [GO:0043588]; T-helper cell differentiation [GO:0042093]; tryptophan metabolic process [GO:0006568]
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GO - Molecular function (MF):
ATP binding [GO:0005524]; ATP hydrolysis activity [GO:0016887]; chaperone binding [GO:0051087]; copper-dependent protein binding [GO:0032767]; copper ion binding [GO:0005507]; copper ion transmembrane transporter activity [GO:0005375]; cuprous ion binding [GO:1903136]; P-type divalent copper transpo...
ATP binding [GO:0005524]; ATP hydrolysis activity [GO:0016887]; chaperone binding [GO:0051087]; copper-dependent protein binding [GO:0032767]; copper ion binding [GO:0005507]; copper ion transmembrane transporter activity [GO:0005375]; cuprous ion binding [GO:1903136]; P-type divalent copper transporter activity [GO:0043682]; P-type monovalent copper transporter activity [GO:0140581]; small GTPase binding [GO:0031267]; superoxide dismutase copper chaperone activity [GO:0016532]
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GO - Cellular component (CC):
apical plasma membrane [GO:0016324]; axon [GO:0030424]; basolateral plasma membrane [GO:0016323]; brush border membrane [GO:0031526]; cell leading edge [GO:0031252]; cytosol [GO:0005829]; dendrite [GO:0030425]; early endosome membrane [GO:0031901]; endoplasmic reticulum [GO:0005783]; Golgi apparatus...
apical plasma membrane [GO:0016324]; axon [GO:0030424]; basolateral plasma membrane [GO:0016323]; brush border membrane [GO:0031526]; cell leading edge [GO:0031252]; cytosol [GO:0005829]; dendrite [GO:0030425]; early endosome membrane [GO:0031901]; endoplasmic reticulum [GO:0005783]; Golgi apparatus [GO:0005794]; integral component of membrane [GO:0016021]; late endosome [GO:0005770]; melanosome membrane [GO:0033162]; membrane [GO:0016020]; membrane raft [GO:0045121]; microvillus [GO:0005902]; neuronal cell body [GO:0043025]; neuron projection [GO:0043005]; nucleus [GO:0005634]; perikaryon [GO:0043204]; perinuclear region of cytoplasm [GO:0048471]; phagocytic vesicle membrane [GO:0030670]; plasma membrane [GO:0005886]; postsynaptic density [GO:0014069]; secretory granule [GO:0030141]; trans-Golgi network [GO:0005802]; trans-Golgi network membrane [GO:0032588]; trans-Golgi network transport vesicle [GO:0030140]
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ATP7A
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Kaplan plot for ATP7A in
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*NOTE: the Kaplan plots were collected from OncoLnc