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Review
. 2017 Jan;1862(1):3-7.
doi: 10.1016/j.bbalip.2016.08.010. Epub 2016 Aug 21.

Biosynthesis, remodeling and turnover of mitochondrial cardiolipin

Michael Schlame  1 , Miriam L Greenberg  2
Affiliations
Review

Biosynthesis, remodeling and turnover of mitochondrial cardiolipin

Michael Schlame et al. Biochim Biophys Acta Mol Cell Biol Lipids. 2017 Jan.

Abstract

Among mitochondrial lipids, cardiolipin occupies a unique place. It is the only phospholipid that is specific to mitochondria and although it is merely a minor component, accounting for 10-20% of the total phospholipid content, cardiolipin plays an important role in the molecular organization, and thus the function of the cristae. This review covers the formation of cardiolipin, a phospholipid dimer containing two phosphatidyl residues, and its assembly into mitochondrial membranes. While a large body of literature exists on this topic, the review focuses on papers that appeared in the past three years. This article is part of a Special Issue entitled: Lipids of Mitochondria edited by Guenther Daum.

Keywords: Lipids; Membrane assembly; Metabolism; Mitochondria.

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Figures

Figure 1
Figure 1. Cardiolipin biosynthesis in mitochondria
Glycero-3-phosphate (G3P) is acylated to lysophosphatidic acid (LPA) and then to phosphatidic acid (PA). PA formation occurs on the outer face of the outer mitochondrial membrane (OM) and in the endoplasmic reticulum. PA is transferred from the outer to the inner mitochondrial membrane (IM) via the intermembrane space (IMS) and is converted to CL on the matrix face of the IM via the intermedates CDPdiacylglycerol (CDP-DG), phosphatidylglycerophosphate (PGP), and phosphatidylglycerol (PG). The final step of the pathway is the remodeling of nascent cardiolipin (CLn) to mature cardiolipin (CLm). Remodeling occurs in the outer leaflet of the inner membrane and requires disturbance of the bilayer packing order. Mammalian genes are shown for the enzymes of PA synthesis and yeast genes are shown for all other enzymes.
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References

    1. Pineau B, Bourge M, Marion J, Mauve C, Gilard F, Maneta-Peyret L, Moreau P, Satiat-Jeunemaitre B, Brown SC, De Paepe R, Danon A. The importance of cardiolipin synthase for mitochondrial ultrastructure, respiratory function, plant development, and stress responses in Arabidopsis. The Plant cell. 2013;25:4195–4208. - PMC - PubMed
    1. Lin Y, Bogdanov M, Tong S, Guan Z, Zheng L. Substrate Selectivity of Lysophospholipid Transporter LplT Involved in Membrane Phospholipid Remodeling in Escherichia coli. The Journal of biological chemistry. 2016;291:2136–2149. - PMC - PubMed
    1. Hostetler KY, Van den Bosch H, Van Deenen LL. Biosynthesis of cardiolipin in liver mitochondria. Biochimica et biophysica acta. 1971;239:113–119. - PubMed
    1. Schlame M, Haldar D. Cardiolipin is synthesized on the matrix side of the inner membrane in rat liver mitochondria. The Journal of biological chemistry. 1993;268:74–79. - PubMed
    1. Tamura Y, Harada Y, Nishikawa S, Yamano K, Kamiya M, Shiota T, Kuroda T, Kuge O, Sesaki H, Imai K, Tomii K, Endo T. Tam41 is a CDP-diacylglycerol synthase required for cardiolipin biosynthesis in mitochondria. Cell metabolism. 2013;17:709–718. - PMC - PubMed

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