Reports

Aconitase Couples Metabolic Regulation to Mitochondrial DNA Maintenance

Xin Jie Chen, Xiaowen Wang, Brett A. Kaufman,^* Ronald A. Butow

Mitochondrial DNA (mtDNA) is essential for cells to maintain respiratory competency and is inherited as a protein-DNA complex called the nucleoid. We have identified 22 mtDNA-associated proteins in yeast, among which is mitochondrial aconitase (Aco1p). We show that this Krebs-cycle enzyme is essential for mtDNA maintenance independent of its catalytic activity. Regulation of ACO1 expression by the HAP and retrograde metabolic signaling pathways directly affects mtDNA maintenance. When constitutively expressed, Aco1p can replace the mtDNA packaging function of the high-mobility-group protein Abf2p. Thus, Aco1p may integrate metabolic signals and mtDNA maintenance.

Department of Molecular Biology, University of Texas Southwestern Medical Center, 6000 Harry Hines Boulevard, Dallas, TX 75390–9148, USA.

^* Present address: Montreal Neurological Institute, 3801 University, Montreal QC H3A 2B4, Canada.

To whom correspondence should be addressed. E-mail: ronald.butow{at}utsouthwestern.edu

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X. J. Chen, X. Wang, and R. A. Butow (2007)
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J. Biol. Chem. 281, 16935-16942
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Manganese Is the Link between Frataxin and Iron-Sulfur Deficiency in the Yeast Model of Friedreich Ataxia.

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PNAS 103, 1382-1387
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