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 language | English (US) |
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Pages (from-to) | 1-12 |
Number of pages | 12 |
Journal | Journal of Membrane Biology |
Volume | 191 |
Issue number | 1 |
DOIs | |
State | Published - Jan 1 2003 |
Externally published | Yes |
Keywords
- ATP7A
- ATP7B
- Copper
- Menkes disease
- Regulation
- Trafficking
- Transport
- Wilson's disease
ASJC Scopus subject areas
- Biophysics
- Physiology
- Cell Biology