Function and regulation of the mammalian copper-transporting ATPases: insights from biochemical and cell biological approaches

J Membr Biol. 2003 Jan 1;191(1):1-12. doi: 10.1007/s00232-002-1040-6.


Copper is an essential trace element that plays a very important role in cell physiology. In humans, disruption of normal copper homeostasis leads to severe disorders, such as Menkes disease and Wilson's disease. Recent genetic, cell biological, and biochemical studies have begun to dissect the molecular mechanisms involved in transmembrane transport and intracellular distribution of copper in mammalian cells. In this review, we summarize the advances that have been made in understanding of structure, function, and regulation of the key human copper transporters, the Menkes disease and Wilson's disease proteins.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adenosine Triphosphatases / chemistry*
  • Adenosine Triphosphatases / genetics
  • Adenosine Triphosphatases / metabolism*
  • Amino Acid Sequence
  • Animals
  • Biological Transport, Active / physiology
  • Cation Transport Proteins / chemistry*
  • Cation Transport Proteins / genetics
  • Cation Transport Proteins / metabolism*
  • Copper / metabolism*
  • Copper-Transporting ATPases
  • Hepatolenticular Degeneration / genetics
  • Hepatolenticular Degeneration / metabolism*
  • Homeostasis*
  • Humans
  • Mammals
  • Menkes Kinky Hair Syndrome / genetics
  • Menkes Kinky Hair Syndrome / metabolism*
  • Molecular Sequence Data
  • Protein Conformation
  • Recombinant Fusion Proteins*
  • Structure-Activity Relationship


  • Cation Transport Proteins
  • Recombinant Fusion Proteins
  • Copper
  • Adenosine Triphosphatases
  • ATP7A protein, human
  • Copper-Transporting ATPases