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Originally published In Press as doi:10.1074/jbc.M004032200 on June 14, 2000

J. Biol. Chem., Vol. 275, Issue 35, 26780-26785, September 1, 2000
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A Human SCO2 Mutation Helps Define the Role of Sco1p in the Cytochrome Oxidase Assembly Pathway*

Elizabeth K. DickinsonDagger , Denise L. AdamsDagger , Eric A. Schon§, and D. Moira GlerumDagger

From the Dagger  Department of Medical Genetics, University of Alberta, Edmonton, Alberta T6G 2H7, Canada and the § Departments of Neurology and of Genetics and Development, Columbia University, New York, New York 10032

Deficiencies in cytochrome oxidase, the terminal enzyme of the mitochondrial respiratory chain, are most often caused by an inability to complete assembly of the enzyme. Pathogenic mutations in SCO2, which encodes a cytochrome oxidase assembly factor, were recently described in several cases of fatal infantile cardioencephalomyopathy. To determine the molecular etiology of these disorders, we describe the generation and characterization of the parallel mutations in the homologous yeast SCO1 gene. We show that the E155K yeast sco1 mutant is respiration-competent, whereas the S240F mutant is not. Interestingly, the S240F mutation allows partial but incorrect assembly of cytochrome oxidase, as judged by an altered cytochrome aa3 peak. Immunoblot analysis reveals a specific absence of subunit 2 from the cytochrome oxidase in this mutant. Taken together, our data suggest that Sco1p provides copper to the CuA site on subunit 2 at a step occurring late in the assembly pathway. This is the first instance of a yeast cytochrome oxidase assembly mutant that is partially assembled. The S240F mutant also represents a powerful new tool with which to elucidate further steps in the cytochrome oxidase assembly pathway.

* This work was supported by grants from the Medical Research Council (MRC) of Canada and the Alberta Heritage Foundation for Medical Research (AHFMR).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.

An MRC and AHFMR Scholar. To whom correspondence should be addressed: Dept. of Medical Genetics, University of Alberta, 8-33 Medical Sciences Bldg., Edmonton, Alberta T6G 2H7, Canada. Tel.: 780-492-4563; Fax: 780-492-1998; E-mail: moira.glerum@ualberta.ca.

Copyright © 2000 by The American Society for Biochemistry and Molecular Biology, Inc.
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