Article
Disruption of copper homeostasis due to a mutation of Atp7a delays the onset of prion disease.
Proceedings of the National Academy of Sciences of the United States of America - 21 Aug 2012
Siggs Owen M, Cruite Justin T, Du Xin, Rutschmann Sophie, Masliah Eliezer, Beutler Bruce, Oldstone Michael B A
Abstract excerpt
Copper influences the pathogenesis of prion disease, but whether it is beneficial or detrimental remains controversial. Copper homeostasis is also essential for normal physiology, as highlighted by the spectrum of diseases caused by disruption of the copper transporting enzymes ATP7A and ATP7B. Here, by using a forward genetics approach in mice, we describe the isolation of three alleles of Atp7a, each with...
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