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• W2118480909 endingPage "1046" @default.
• W2118480909 startingPage "1011" @default.
• W2118480909 abstract "Copper-transporting ATPases (Cu-ATPases) ATP7A and ATP7B are evolutionarily conserved polytopic membrane proteins with essential roles in human physiology. The Cu-ATPases are expressed in most tissues, and their transport activity is crucial for central nervous system development, liver function, connective tissue formation, and many other physiological processes. The loss of ATP7A or ATP7B function is associated with severe metabolic disorders, Menkes disease, and Wilson disease. In cells, the Cu-ATPases maintain intracellular copper concentration by transporting copper from the cytosol across cellular membranes. They also contribute to protein biosynthesis by delivering copper into the lumen of the secretory pathway where metal ion is incorporated into copper-dependent enzymes. The biosynthetic and homeostatic functions of Cu-ATPases are performed in different cell compartments; targeting to these compartments and the functional activity of Cu-ATPase are both regulated by copper. In recent years, significant progress has been made in understanding the structure, function, and regulation of these essential transporters. These studies raised many new questions related to specific physiological roles of Cu-ATPases in various tissues and complex mechanisms that control the Cu-ATPase function. This review summarizes current data on the structural organization and functional properties of ATP7A and ATP7B as well as their localization and functions in various tissues, and discusses the current models of regulated trafficking of human Cu-ATPases." @default.
• W2118480909 created "2016-06-24" @default.
• W2118480909 creator A5014109466 @default.
• W2118480909 creator A5036328192 @default.
• W2118480909 creator A5062797581 @default.
• W2118480909 creator A5079179187 @default.
• W2118480909 date "2007-07-01" @default.
• W2118480909 modified "2023-10-10" @default.
• W2118480909 title "Function and Regulation of Human Copper-Transporting ATPases" @default.
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