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Legend: Although mitochondrial membranes contain phosphatidylcholine (PC), phosphatidylethanolamine (PE), phosphatidylinositol (PI) and cardiolipin (CL) as major phospholipids, PC, PE and PI are mainly synthesized in the ER and transported to mitochondria. Phosphatidylserine (PS) synthesized in the ER is also transported to mitochondria and is used for biosynthesis of PE by PS decarboxylase. It has been suggested that CL is synthesized de novo in mitochondria from glycerol 3-phosphate and acyl-ACP. However, a part of PG and CDP-DAG synthesized in the ER might be transported to mitochondria and used for biosynthesis of CL.
Abbreviations: AG3P, acylglycerol 3-phosphate; ALCAT, acyl-CoA: monolysocardiolipin acyltransferase; CDP-DAG, CDP-diacylglycerol; CDP-DAGS, CDP-diacylglycerol synthase; CLD, cardiolipin deacylase; CLS, cardiolipin synthase; DAG3P, diacylglycerol 3-phosphate; GPAT, glycerol-3-phosphate acyltransferase; LPAAT, lysophosphatidic acid acyltransferase; PGP, phosphatidylglycerolphosphate; PGPP, phosphatidylglycerol-phosphate phosphatase; PGPS, phosphatidylglycerol-phosphate synthase; PSD, phosphatidylserine decarboxylase; TAZ, Taffazzin (cardiolipin transacylase); VLCFA, very long chain fatty acid. Mitochondrial Phospholipid Synthesis
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