Molecular and cellular aspects of copper transport in developing mammals

Mercer, Julian and Llanos, Roxana 2003, Molecular and cellular aspects of copper transport in developing mammals, Journal of nutrition, vol. 133, no. 5/Supplement, pp. 1481S-1484S.

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Title Molecular and cellular aspects of copper transport in developing mammals
Author(s) Mercer, Julian
Llanos, Roxana
Journal name Journal of nutrition
Volume number 133
Issue number 5/Supplement
Start page 1481S
End page 1484S
Publisher American Society for Clinical Nutrition
Place of publication Bethesda, Md.
Publication date 2003-05
ISSN 0022-3166
Keyword(s) copper
Menkes disease
Wilson disease
Summary Copper is an essential trace element that requires tightly regulated homeostatic mechanisms to ensure adequate supplies without any toxic effects because of the ability of the metal ion to catalyze the formation of free radicals. The Cu-ATPases, ATP7A and ATP7B, play an important role in the physiological regulation of copper. Adequate supplies of copper are particularly important in developing animals, and in humans this is illustrated by mutations of ATP7A that cause the copper deficiency condition Menkes disease, which is fatal in early childhood. In contrast, mutations in ATP7B result in the genetic toxicosis, Wilson disease. We propose that the physiological regulation of copper is accomplished mainly by the intracellular copper-regulated trafficking of the Cu-ATPases. This process allows the overall copper status in the body to be maintained when levels of copper in the diet alter. A study of the defects in mouse models of Menkes and Wilson diseases has demonstrated that both ATPases play an important role in supplying copper to the developing fetus and neonate
Notes Supplement: 11th International Symposium on Trace Elements in Man and Animals
Language eng
Field of Research 060403 Developmental Genetics (incl Sex Determination)
Socio Economic Objective 970106 Expanding Knowledge in the Biological Sciences
HERDC Research category C1 Refereed article in a scholarly journal
Copyright notice ©2003, American Society for Nutritional Sciences
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Document type: Journal Article
Collection: School of Biological and Chemical Sciences
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