The Menkes and Wilson disease genes counteract in copper toxicosis in Labrador retrievers: a new canine model for copper-metabolism disorders

The Menkes and Wilson disease genes counteract in copper toxicosis in Labrador retrievers: a new canine model for copper-metabolism disorders

The deleterious effects of a disrupted copper metabolism are illustrated by hereditary diseases caused by mutations in the genes coding for the copper transporters ATP7A and ATP7B. Menkes disease, involving ATP7A, is a fatal neurodegenerative disorder of copper deficiency. Mutations in ATP7B lead to...

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Bibliographic Details
Main Authors: Hille Fieten, Yadvinder Gill, Alan J. Martin, Mafalda Concilli, Karen Dirksen, Frank G. van Steenbeek, Bart Spee, Ted S. G. A. M. van den Ingh, Ellen C. C. P. Martens, Paola Festa, Giancarlo Chesi, Bart van de Sluis, Roderick H. J. H. Houwen, Adrian L. Watson, Yurii S. Aulchenko, Victoria L. Hodgkinson, Sha Zhu, Michael J. Petris, Roman S. Polishchuk, Peter A. J. Leegwater, Jan Rothuizen
Format: Article
Language:English
Published: The Company of Biologists 2016-01-01
Series:Disease Models & Mechanisms
Subjects:
ATP7A
ATP7B
Dog
Liver
Online Access:http://dmm.biologists.org/content/9/1/25

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