期刊
ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS
卷 476, 期 1, 页码 22-32出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.abb.2008.05.005
关键词
ATP7A; ATP7B; P-type ATPase; copper; transport; trafficking
资金
- NIDDK NIH HHS [R01 DK071865, R01 DK071865-03] Funding Source: Medline
The human copper-transporting ATPases (Cu-ATPases) are essential for dietary copper uptake, normal development and function of the CNS, and regulation of copper homeostasis in the body. In a cell, Cu-ATPases maintain the intracellular concentration of copper by transporting copper into intracellular exocytic vesicles. In addition, these P-type ATPases mediate delivery of copper to copper-dependent enzymes in the secretory pathway and in specialized cell compartments such as secretory granules or melanosomes. The multiple functions of human Cu-ATPase necessitate complex regulation of these transporters that is mediated through the presence of regulatory domains in their structure, posttranslational modification and intracellular trafficking, as well as interactions with the copper chaperone Atox1 and other regulatory molecules. In this review, we summarize the current information on the function and regulatory mechanisms acting on human Cu-ATPases ATP7A and ATP7B. Brief comparison with the Cu-ATPase orthologs from other species is included. (c) 2008 Elsevier Inc. All rights reserved.
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