The Mitochondrial Metallochaperone SCO1 Is Required to Sustain Expression of the High-Affinity Copper Transporter CTR1 and Preserve Copper Homeostasis

Human SCO1 fulfills essential roles in cytochrome c oxidase (COX) assembly and the regulation of copper (Cu) homeostasis, yet it remains unclear why pathogenic mutations in this gene cause such clinically heterogeneous forms of disease. Here, we establish a Sco1 mouse model of human disease and show...

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Bibliographic Details
Main Authors: Christopher J. Hlynialuk, Binbing Ling, Zakery N. Baker, Paul A. Cobine, Lisa D. Yu, Aren Boulet, Timothy Wai, Amzad Hossain, Amr M. El Zawily, Pamela J. McFie, Scot J. Stone, Francisca Diaz, Carlos T. Moraes, Deepa Viswanathan, Michael J. Petris, Scot C. Leary
Format: Article
Language:English
Published: Elsevier 2015-02-01
Series:Cell Reports
Online Access:http://www.sciencedirect.com/science/article/pii/S2211124715000327