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J. Biol. Chem., Vol. 281, Issue 6, 3743-3751, February 10, 2006

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COX24 Codes for a Mitochondrial Protein Required for Processing of the COX1 Transcript^*

From the Department of Biological Sciences, Columbia University, New York, New York 10027

In most strains of Saccharomyces cerevisiae the mitochondrial gene COX1, for subunit 1 of cytochrome oxidase, contains multiple exons and introns. Processing of COX1 primary transcript requires accessory proteins factors, some of which are encoded by nuclear genes and others by reading frames residing in some of the introns of the COX1 and COB genes. Here we show that the low molecular weight protein product of open reading frame YLR204W, for which we propose the name COX24, is also involved in processing of COX1 RNA intermediates. The growth defect of cox24 mutants is partially rescued in strains harboring mitochondrial DNA lacking introns. Northern blot analyses of mitochondrial transcripts indicate cox24 null mutants to be blocked in processing of introns aI2 and aI3. The dependence of intron aI3 excision on Cox24p is also supported by the growth properties of the cox24 mutant harboring mitochondrial DNA with different intron compositions. The intermediate phenotype of the cox24 mutant in the background of intronless mitochondrial DNA, however, suggests that in addition to its role in splicing of the COX1 pre-mRNA, Cox24p still has another function. Based on the analysis of a cox14-cox24 double mutant, we propose that the other function of Cox24p is related to translation of the COX1 mRNA.

Received for publication, October 3, 2005 , and in revised form, December 1, 2005.

^* This work was supported in part by National Institutes of Health Research Grant GM45952. The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

¹ Supported by a grant from the Conselho Nacional Desenvolvimento Cientifico e Tecnologico (CNPq) Fundação Amparo a Pesquisa São Paulo (FAPESP). Current address: Dept. of Genetics, IBB-UNESP, Botucatu/SP 18607-741, Brazil.

² Current address: Dept. of Biochemistry, Biophysics and Molecular Biology, Iowa State University, Ames, IA 50011.

³ To whom correspondence should be addressed. Tel.: 212-854-2920; Fax: 212-856-8246; E-mail: spud{at}cubpet2.bio.columbia.edu.

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