Trafficking of the copper-ATPases, ATP7A and ATP7B: Role in copper homeostasis

La Fontaine, Sharon and Mercer, Julian 2007, Trafficking of the copper-ATPases, ATP7A and ATP7B: Role in copper homeostasis, Archives of biochemistry and biophysics, vol. 463, no. 2, pp. 149-167, doi: 10.1016/

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Title Trafficking of the copper-ATPases, ATP7A and ATP7B: Role in copper homeostasis
Author(s) La Fontaine, SharonORCID iD for La Fontaine, Sharon
Mercer, Julian
Journal name Archives of biochemistry and biophysics
Volume number 463
Issue number 2
Start page 149
End page 167
Publisher Elsevier
Place of publication Amsterdam, The Netherlands
Publication date 2007-07-15
ISSN 0003-9861
Keyword(s) copper homeostasis
P-type ATPase
Menkes disease
Wilson disease
Summary Copper is essential for human health and copper imbalance is a key factor in the aetiology and pathology of several neurodegenerative diseases. The copper-transporting P-type ATPases, ATP7A and ATP7B are key molecules required for the regulation and maintenance of mammalian copper homeostasis. Their absence or malfunction leads to the genetically inherited disorders, Menkes and Wilson diseases, respectively. These proteins have a dual role in cells, namely to provide copper to essential cuproenzymes and to mediate the excretion of excess intracellular copper. A unique feature of ATP7A and ATP7B that is integral to these functions is their ability to sense and respond to intracellular copper levels, the latter manifested through their copper-regulated trafficking from the transGolgi network to the appropriate cellular membrane domain (basolateral or apical, respectively) to eliminate excess copper from the cell. Research over the last decade has yielded significant insight into the enzymatic properties and cell biology of the copper-ATPases. With recent advances in elucidating their localization and trafficking in human and animal tissues in response to physiological stimuli, we are progressing rapidly towards an integrated understanding of their physiological significance at the level of the whole animal. This knowledge in turn is helping to clarify the biochemical and cellular basis not only for the phenotypes conferred by individual Menkes and Wilson disease patient mutations, but also for the clinical variability of phenotypes associated with each of these diseases. Importantly, this information is also providing a rational basis for the applicability and appropriateness of certain diagnostic markers and therapeutic regimes. This overview will provide an update on the current state of our understanding of the localization and trafficking properties of the copper-ATPases in cells and tissues, the molecular signals and posttranslational interactions that govern their trafficking activities, and the cellular basis for the clinical phenotypes associated with disease-causing mutations.
Language eng
DOI 10.1016/
Field of Research 060199 Biochemistry and Cell Biology not elsewhere classified
HERDC Research category C1 Refereed article in a scholarly journal
Copyright notice ©2007, Elsevier Inc.
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