Cellular copper transport and metabolism.
The transport and cellular metabolism of Cu depends on a series of membrane proteins and smaller soluble peptides that comprise a functionally integrated system for maintaining cellular Cu homeostasis. Inward transport across the plasma membrane appears to be a function of integral membrane proteins that form the channels that select Cu ions for passage. Two membrane-bound Cu-transporting ATPase enzymes, ATP7A and ATP7B, the products of the Menkes and Wilson disease genes, respectively, catalyze an ATP-dependent transfer of Cu to intracellular compartments or expel Cu from the cell. ATP7A and ATP7B work in concert with a series of smaller peptides, the copper chaperones, that exchange Cu at the ATPase sites or incorporate the Cu directly into the structure of Cu-dependent enzymes such as cytochrome c oxidase and Cu, Zn superoxide dismutase. These mechanisms come into play in response to a high influx of Cu or during the course of normal Cu metabolism.
Harris
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Department of Biochemistry and Biophysics and the Faculty of Nutrition, Texas A&M University, College Station, Texas 77843-2128, USA. eharris@tamu.edu
D000251:Adenosine Triphosphatases / Q000235:genetics / Q000378:metabolism; D000418:Albumins / Q000502:physiology; D001692:Biological Transport; D002352:Carrier Proteins / Q000502:physiology*; D002570:Ceruloplasmin / Q000502:physiology; D003300:Copper / Q000378:metabolism* / Q000502:physiology / Q000627:therapeutic use; D005978:Glutathione / Q000502:physiology; D006527:Hepatolenticular Degeneration / Q000235:genetics / Q000378:metabolism; D008099:Liver / Q000378:metabolism*; D007706:Menkes Kinky Hair Syndrome / Q000235:genetics / Q000378:metabolism
D000418:Albumins; D002352:Carrier Proteins; D003300:Copper; D002570:Ceruloplasmin; D000251:Adenosine Triphosphatases; D005978:Glutathione