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Originally published In Press as doi:10.1074/jbc.M211413200 on January 14, 2003

J. Biol. Chem., Vol. 278, Issue 14, 12278-12284, April 4, 2003
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Supplying Copper to the Cuproenzyme Peptidylglycine alpha -Amidating Monooxygenase*

Rajaâ El MeskiniDagger §, Valeria Cizewski Culotta, Richard E. MainsDagger , and Betty A. EipperDagger ||

From the Dagger  Department of Neuroscience, University of Connecticut Health Center, Farmington, Connecticut 06030-3401 and  Department of Environmental Health Sciences, The Johns Hopkins University School of Public Health, Baltimore, Maryland 21205

We explored the role of known copper transporters and chaperones in delivering copper to peptidylglycine-alpha -hydroxylating monooxygenase (PHM), a copper-dependent enzyme that functions in the secretory pathway lumen. We examined the roles of yeast Ccc2, a P-type ATPase related to human ATP7A (Menkes disease protein) and ATP7B (Wilson disease protein), as well as yeast Atx1, a cytosolic copper chaperone. We expressed soluble PHMcc (catalytic core) in yeast using the yeast pre-pro-alpha -mating factor leader region to target the enzyme to the secretory pathway. Although the yeast genome encodes no PHM-like enzyme, PHMcc expressed in yeast is at least as active as PHMcc produced by mammalian cells. PHMcc partially co-migrated with a Golgi marker during subcellular fractionation and partially co-localized with Ccc2 based on immunofluorescence. To determine whether production of active PHM was dependent on copper trafficking pathways involving the CCC2 or ATX1 genes, we expressed PHMcc in wild-type, ccc2, and atx1 mutant yeast. Although ccc2 and atx1 mutant yeast produce normal levels of PHMcc protein, it lacks catalytic activity. Addition of exogenous copper yields fully active PHMcc. Similarly, production of active PHM in mouse fibroblasts is impaired in the presence of a mutant ATP7A gene. Although delivery of copper to lumenal cuproproteins like PAM involves ATP7A, lumenal chaperones may not be required.

* This work was supported in part by National Institutes of Health Grants DK-32949 (to B. A. E.) and GM-50016 (to V. C. C.).The costs of publication of this article were defrayed in part by the payment of page charges. The article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

§ Supported in part by a research grant from Roche Diagnostics (Penzberg, Germany).

|| To whom correspondence should be addressed: Dept. of Neuroscience, University of Connecticut Health Center, 263 Farmington Ave., Farmington, CT 06030-3401. Tel.: 860-679-8898; Fax: 860-679-1885; E-mail: eipper@uchc.edu.

Copyright © 2003 by The American Society for Biochemistry and Molecular Biology, Inc.
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