Journal
JOURNAL OF MEMBRANE BIOLOGY
Volume 191, Issue 1, Pages 1-12Publisher
SPRINGER-VERLAG
DOI: 10.1007/s00232-002-1040-6
Keywords
copper; Menkes disease; Wilson's disease; ATP7A; ATP7B; transport; trafficking; regulation
Funding
- NIDDK NIH HHS [R01-DK59893, R01-DK55719] Funding Source: Medline
- NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES [R01DK059893, R01DK055719] Funding Source: NIH RePORTER
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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.
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