Abstract
Mitochondrial membranes have a unique lipid composition necessary for proper shape and function of the organelle. Mitochondrial lipid metabolism involves biosynthesis of the phospholipids phosphatidylethanolamine, cardiolipin and phosphatidylglycerol, the latter is a precursor of the late endosomal lipid bis(monoacylglycero)phosphate. It also includes mitochondrial fatty acid synthesis necessary for the formation of the lipid cofactor lipoic acid. Furthermore the synthesis of coenzyme Q takes place in mitochondria as well as essential parts of the steroid and vitamin D metabolism. Lipid transport and remodelling, which are necessary for tailoring and maintaining specific membrane properties, are just partially unravelled. Mitochondrial lipids are involved in organelle maintenance, fission and fusion, mitophagy and cytochrome c-mediated apoptosis. Mutations in TAZ, SERAC1 and AGK affect mitochondrial phospholipid metabolism and cause Barth syndrome, MEGDEL and Sengers syndrome, respectively. In these disorders an abnormal mitochondrial energy metabolism was found, which seems to be due to disturbed protein-lipid interactions, affecting especially enzymes of the oxidative phosphorylation. Since a growing number of enzymes and transport processes are recognised as parts of the mitochondrial lipid metabolism, a further increase of lipid-related disorders can be expected.
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Supported by the E-Rare project GENOMIT FWF I 920-B13 and the Vereinigung zur Förderung Pädiatrischer Forschung und Fortbildung Salzburg.
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Presented at the workshop “Diagnostic Approach, and Classification of IEM Affecting the Synthesis and Catabolism of Complex Lipids” in Paris, France, June 14-15, 2013.
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Mayr, J.A. Lipid metabolism in mitochondrial membranes. J Inherit Metab Dis 38, 137–144 (2015). https://doi.org/10.1007/s10545-014-9748-x
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DOI: https://doi.org/10.1007/s10545-014-9748-x