title: Advances in the understanding of mammalian copper transporters.
abstract: Copper (Cu) is an essential micronutrient. Its ability to exist in 2 oxidation states (Cu(1+) and Cu(2+)) allows it to function as an enzymatic cofactor in hydrolytic, electron transfer, and oxygen utilization reactions. Cu transporters CTR1, ATP7A, and ATP7B play key roles in ensuring that adequate Cu is available for Cu-requiring processes and the prevention of excess Cu accumulation within cells. Two diseases of Cu metabolism, Menkes disease and Wilson disease, which are caused by mutations in ATP7A and ATP7B, respectively, exemplify the critical importance of regulating Cu balance in humans. Herein, we review recent studies of the biochemical and cell biological characteristics of CTR1, ATP7A, and ATP7B, as well as emerging roles for Cu in new areas of physiology.
authors.affiliation: Department of Biochemistry, University of Missouri, Columbia, MO 65211, USA.
authors.forename: Yanfang
authors.initials: Y
authors.lastname: Wang
authors.forename: Victoria
authors.initials: V
authors.lastname: Hodgkinson
authors.forename: Sha
authors.initials: S
authors.lastname: Zhu
authors.forename: Gary A
authors.initials: GA
authors.lastname: Weisman
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Petris
mesh_terms: D000251:Adenosine Triphosphatases / Q000235:genetics / Q000378:metabolism / Q000502:physiology; D000818:Animals; D001692:Biological Transport; D027682:Cation Transport Proteins / Q000235:genetics / Q000378:metabolism* / Q000502:physiology; D003300:Copper / Q000378:metabolism*; D000079923:Copper Transporter 1; D000073840:Copper-Transporting ATPases; D004847:Epithelial Cells / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000235:genetics; D006801:Humans; D008322:Mammals; D007706:Menkes Kinky Hair Syndrome / Q000235:genetics; D000080083:SLC31 Proteins; D014131:Trace Elements / Q000378:metabolism*
chemical_list: D027682:Cation Transport Proteins; D000079923:Copper Transporter 1; D000080083:SLC31 Proteins; C490079:SLC31A1 protein, human; C524078:SLC31A2 protein, human; D014131:Trace Elements; D003300:Copper; D000251:Adenosine Triphosphatases; C081184:ATP7A protein, human; C000612464:ATP7B protein, human; D000073840:Copper-Transporting ATPases