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

S. Lutsenko, M. J. Petris

Research output: Contribution to journalReview article

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1-12
Number of pages12
JournalJournal of Membrane Biology
Volume191
Issue number1
DOIs
StatePublished - Jan 1 2003
Externally publishedYes

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Keywords

  • ATP7A
  • ATP7B
  • Copper
  • Menkes disease
  • Regulation
  • Trafficking
  • Transport
  • Wilson's disease

ASJC Scopus subject areas

  • Biophysics
  • Physiology
  • Cell Biology

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