Alteration of copper physiology in mice overexpressing the human menkes protein ATP7A

Ke, Bi-Xia, Llanos, Roxana, Wright, Magali, Deal, Yolanda and Mercer, Julian 2006, Alteration of copper physiology in mice overexpressing the human menkes protein ATP7A, American journal of physiology : regulatory, interative comparative physiology, vol. 290, pp. 1460-1467, doi: 10.1152/ajpregu.00806.2005.

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Title Alteration of copper physiology in mice overexpressing the human menkes protein ATP7A
Author(s) Ke, Bi-Xia
Llanos, Roxana
Wright, Magali
Deal, Yolanda
Mercer, Julian
Journal name American journal of physiology : regulatory, interative comparative physiology
Volume number 290
Start page 1460
End page 1467
Publisher American Physiological Society
Place of publication Bethesda, Md.
Publication date 2006-01-05
ISSN 0363-6119
Keyword(s) copper homeostasis
transgenic mice
Summary The Menkes protein (ATP7A) is defective in the Cu deficiency disorder Menkes disease and is an important contributor to the maintenance of physiological Cu homeostasis. To investigate more fully the role of ATP7A, transgenic mice expressing the human Menkes gene ATP7A from chicken beta-actin composite promoter (CAG) were produced. The transgenic mice expressed ATP7A in lung, heart, liver, kidney, small intestine, and brain but displayed no overt phenotype resulting from expression of the human protein. Immunohistochemical analysis revealed that ATP7A was found primarily in the cardiac muscle, smooth muscle of the lung, distal tubules of the kidney, intestinal enterocytes, and patches of hepatocytes, as well as in the hippocampus, cerebellum, and choroid plexus of the brain. In 60-day- and 300-day-old mice, Cu concentrations were reduced in most tissues, consistent with ATP7A playing a role in Cu efflux. The reduction in Cu was most pronounced in the hearts of older T22#2 females (24%), T22#2 males (18%), and T25#5 females (23%), as well as in the brains of 60-day-old T22#2 females and males (23% and 30%, respectively).
Language eng
DOI 10.1152/ajpregu.00806.2005
Field of Research 060603 Animal Physiology - Systems
HERDC Research category C1 Refereed article in a scholarly journal
ERA Research output type C Journal article
Copyright notice ©2006, American Physiological Society
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Document type: Journal Article
Collection: School of Life and Environmental Sciences
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