EMBO J. 1991 Aug;10(8):2023-31.

ABC1, a novel yeast nuclear gene has a dual function in mitochondria: it suppresses a cytochrome b mRNA translation defect and is essential for the electron transfer in the bc 1 complex.

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Centre de Génétique Moléculaire, Laboratoire propre du CNRS associé à l'Université Pierre et Marie Curie, Gif-sur-Yvette, France.

Abstract

We have cloned and sequenced a novel yeast nuclear gene ABC1 which suppresses, in multicopy, the cytochrome b mRNA translation defect due to the nuclear mutation cbs2-223. Analysis of the ABC1 gene shows that it is weakly expressed, it could code for a protein of 501 amino acids which has a typical presequence of a protein imported into mitochondria and which does not display a strong similarity to any known protein. Inactivation of the ABC1 gene is not lethal to the cell but leads to a respiratory defect: no oxygen uptake and no growth on non-fermentable media. A total absence of NADH-cytochrome c oxidoreductase and succinate-cytochrome c oxidoreductase activities concomitant with the presence of specific dehydrogenases, suggests a block in the bc 1 segment of the respiratory chain. However, all the cytochromes are spectrally detectable. Cytochrome b is quite efficiently reduced while cytochromes c1 and c are not. The function of ABC1 in the suppression of a defect in apocytochrome b mRNA translation and in the activity of the bc1 complex suggests that the ABC1 protein would be a novel chaperonin involved both in biogenesis and bioenergetics of mitochondria.

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PMCID:: PMC452883

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The nuclear ABC1 gene is essential for the correct conformation and functioning of the cytochrome bc1 complex and the neighbouring complexes II and IV in the mitochondrial respiratory chain. [Eur J Biochem. 1997]
The nuclear ABC1 gene is essential for the correct conformation and functioning of the cytochrome bc1 complex and the neighbouring complexes II and IV in the mitochondrial respiratory chain.
Brasseur G, Tron G, Dujardin G, Slonimski PP, Brivet-Chevillotte P. Eur J Biochem. 1997 May 15; 246(1):103-11.
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Review [Complex III deficiency due to mutations in the cytochrome b gene]. [Nihon Rinsho. 2002]
Review [Complex III deficiency due to mutations in the cytochrome b gene].
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ABC1, a novel yeast nuclear gene has a dual function in mitochondria: it suppresses a cytochrome b mRNA translation defect and is essential for the electron transfer in the bc 1 complex.

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