FEATURES

FAMILY

cation transport ATPase (P-type) (TC 3.A.3) family

DESCRIPTION

Also known as ATP7A_HUMAN, ATP7A, MC1, MNK. 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). Monomer. Interacts with PDZD11 (PubMed:16051599). Interacts with ATOX1 and COMMD1 (PubMed:21667063, PubMed:19453293, PubMed:31283225). Interacts with TYRP1 (By similarity). Interacts with SOD3; this interaction is copper-dependent and is required for SOD3 activity.

Also known as ATP7A_HUMAN, ATP7A, MC1, MNK. ATP-driven copper (Cu(+)) ion pump that plays an important role in intracellular copper ion homeostasis (PubMed:10419525, PubMed:11092760, PubMed:28389 ...

EXTERNAL LINKS

ATP7A

Mutations

Mutation(s) distribution

Mutations table