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ATOX1

Domain

The heavy-metal-associated domain (HMA) coordinates a Cu(+) ion via the cysteine residues within the CXXC motif. The transfer of Cu(+) ion from ATOX1 to ATP7A involves the formation of a three-coordinate Cu(+)-bridged heterodimer where the metal is shared between the two metal binding sites of ATOX1 and ATP7A. The Cu(+) ion appears to switch between two coordination modes, forming two links with one protein and one with the other. Cisplatin, a chemotherapeutic drug, can bind the CXXC motif and hinder the release of Cu(+) ion.

Function

Binds and deliver cytosolic copper to the copper ATPase proteins. May be important in cellular antioxidant defense.

Sequence Similarities

Belongs to the ATX1 family.

Tissue Specificity

Ubiquitous.

Alternative names

HAH1, ATOX1, Copper transport protein ATOX1, Metal transport protein ATX1

swissprot:O00244 omim:602270 entrezGene:475