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Molecular basis of copper transport: cellular and physiological functions of Menkes and Wilson disease proteins (ATP7A and ATPB)

Kramer, David, Llanos, Roxana and Mercer, Julian 2003, Molecular basis of copper transport: cellular and physiological functions of Menkes and Wilson disease proteins (ATP7A and ATPB). In Zatta, Paolo (ed), Metal ions and neurodegenerative disorders, World Scientific Publishing, Singapore, pp.207-244.

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Title Molecular basis of copper transport: cellular and physiological functions of Menkes and Wilson disease proteins (ATP7A and ATPB)
Author(s) Kramer, David
Llanos, Roxana
Mercer, Julian
Title of book Metal ions and neurodegenerative disorders
Editor(s) Zatta, Paolo
Publication date 2003
Total chapters 22
Start page 207
End page 244
Total pages 38
Publisher World Scientific Publishing
Place of Publication Singapore
Keyword(s) Menkes disease
Wilson disease
ATPase
Summary Eukaryotic cells prevent copper-induced, free radical damage to cell components by employing copper-binding proteins and transporters that minimize the likelihood of free copper ions existing in the cell. In the cell, copper is actively transported from the cytoplasm during the biosynthesis of secreted coppercontaining proteins and, as a protective measure, when there is an excess of copper. In humans, this is accomplished by two related copper-transporting ATPases (ATP7A and ATP7B), which are the affected genes in two distinct human genetic disorders of copper transport, Menkes disease (copper deficiency) and Wilson disease (copper toxicosis). The study of these ATPases has revealed their molecular mechanisms of copper transport and their roles in physiological copper homeostasis. Both ATP7A and ATP7B are expressed in specific brain regions and neurological abnormalities are important clinical features in Menkes and Wilson disease.
ISBN 9812383980
9789812383983
Language eng
Field of Research 060199 Biochemistry and Cell Biology not elsewhere classified
HERDC Research category B1 Book chapter
Copyright notice ©2003, World Scientific Publishing Co
Persistent URL http://hdl.handle.net/10536/DRO/DU:30000577

Document type: Book Chapter
Collection: School of Biological and Chemical Sciences
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