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. 2020 Oct 28;21(21):8031. doi: 10.3390/ijms21218031

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© 2020 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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Cardiolipin metabolism is a two-step process. Biosynthesis step results in the formation of immature CL with acyl chain composition that is not yet definitive. Fatty acyl chain of immature CL undergoes several modifications through the activity of remodeling proteins, leading to the formation of mature CL. Abbreviations: PA, phosphatidic acid; Tamm41 (CDP-DS), CDP diacylglycerol synthase; CDP-DG, cytidine diphosphate-diacylglycerol; Pgs1, phosphatidylglycerophosphate synthase 1; PGP, phosphatidylglycerol phosphate; PTPMT1 (PGPP), phosphatidylglycerophosphate phosphatase 1; PG, phosphatidylglycerol; CRLS1, cardiolipin synthase 1; CL, cardiolipin; PLA2, phospholipase A2; MLCL, monolysocardiolipin; MLCLAT1, monolysocardiolipin acyltransferase 1; TAZ, tafazzin; ALCAT1, acyl-CoA:lysocardiolipin acyltransferase; OMM, outer mitochondrial membrane; IMM, inner mitochondrial membrane; IMS, intermembrane space; PE, phosphatidylethanolamine; PC, phosphatidylcholine.