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Disruption of copper homeostasis due to a mutation of Atp7a delays the onset of prion disease.

Abstract:

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 different phenotypic consequences. The mildest of the three, Atp7a(brown), was insuffi...

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Publisher copy:
10.1073/pnas.1211499109

Authors


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Institution:
University of Oxford
Department:
Oxford, MSD, NDORMS
Cruite, JT More by this author
Rutschmann, S More by this author
Masliah, E More by this author
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Journal:
Proceedings of the National Academy of Sciences of the United States of America
Volume:
109
Issue:
34
Pages:
13733-13738
Publication date:
2012-08-05
DOI:
EISSN:
1091-6490
ISSN:
0027-8424
URN:
uuid:070bf0d9-a330-4e75-a2e7-9d8df4eaf2a7
Source identifiers:
418814
Local pid:
pubs:418814

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