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Am J Physiol Regul Integr Comp Physiol (January 5, 2006). doi:10.1152/ajpregu.00806.2005
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Submitted on November 15, 2005
Accepted on December 24, 2005

Alteration of Copper Physiology in Mice Over-expressing the Human Menkes Protein ATP7A

Bi-Xia Ke1, Roxana M Llanos1, Magali Wright1, Yolanda Deal1, and Julian F. B Mercer1*

1 Centre for Cellular and Molecular Biology, School of Biological and Chemical Sciences, Deakin University, Melbourne, Victoria, Australia

* To whom correspondence should be addressed. E-mail: jmercer{at}deakin.edu.au.

The Menkes protein (ATP7A) is defective in the Cu deficiency disorder, Menkes disease and is an important contributor to maintenance of physiological copper 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 expressed primarily in the cardiac muscle, smooth muscle of the lung, distal tubules of the kidney, in the intestinal enterocytes, in patches of hepatocytes, and in the 29 hippocampus, cerebellum and choroid plexus of the brain. In 60 day and 300 day mice copper concentrations were reduced in most tissues, consistent with ATP7A playing a role in copper efflux. The reduction in copper was most pronounced in the hearts of older T22#2 females (24%), T22#2 males (18%) and T25#5 females (23%) and in the brains of 60 day old T22#2 females and males (23% and 30%, respectively).




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