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Reference: Hiltunen JK, et al. (2009) Mitochondrial Fatty Acid Synthesis Type II: More than Just Fatty Acids. J Biol Chem 284(14):9011-5

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Abstract


Eukaryotes harbor a highly conserved mitochondrial pathway for fatty acid synthesis (FAS), which is completely independent of the eukaryotic cytosolic apparatus. The activities of the mitochondrial FAS system are catalyzed by soluble enzymes, and thus the pathway resembles its prokaryotic counterparts. Except for octanoic acid, which is the direct precursor for lipoic acid synthesis, other end-products and functions of the mitochondrial FAS pathway are still largely enigmatic. In addition to low cellular levels of lipoic acid, disruption of genes encoding mitochondrial FAS enzymes in yeast results in a respiratory deficient phenotype and small rudimentary mitochondria. Recently, two distinct links between mitochondrial FAS and RNA processing have been discovered in vertebrates and yeast respectively. In vertebrates, the mitochondrial 3-hydroxyacyl-ACP dehydratase and the RPP14 subunit of RNase P are encoded by the same bicistronic transcript in an evolutionary conserved arrangement that is unusual for eukaryotes. In yeast, defects in mitochondrial fatty acid synthesis result in inefficient RNase P cleavage in the organelle. The intersection of mitochondrial FAS and RNA metabolism in both systems provides a novel mechanism for the coordination of intermediary metabolism in eukaryotic cells.

Reference Type
Journal Article
Authors
Hiltunen JK, Schonauer MS, Autio KJ, Mittelmeier TM, Kastaniotis AJ, Dieckmann CL
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