A unique organelle for studying membrane biochemistry is the mitochondrion whose functionality depends on a coordinated supply of proteins and lipids. Mitochondria are capable of synthesizing several lipids autonomously such as phosphatidylglycerol, cardiolipin and in part phosphatidylethanolamine, phosphatidic acid and CDP-diacylglycerol. Other mitochondrial membrane lipids such as phosphatidylcholine, phosphatidylserine, phosphatidylinositol, sterols and sphingolipids have to be imported. The mitochondrial lipid composition, the biosynthesis and the import of mitochondrial lipids as well as the regulation of these processes will be main issues of this review article. Furthermore, interactions of lipids and mitochondrial proteins which are highly important for various mitochondrial processes will be discussed. Malfunction or loss of enzymes involved in mitochondrial phospholipid biosynthesis lead to dysfunction of cell respiration, affect the assembly and stability of the mitochondrial protein import machinery and cause abnormal mitochondrial morphology or even lethality. Molecular aspects of these processes as well as diseases related to defects in the formation of mitochondrial membranes will be described.
Keywords: CDP-diacylglycerol synthase; CDS; CL; CS; DMPE; ER; ER-mitochondria encounter structure; ERMES; IMM; IMS; Lipid synthesis; Lipid transport; MAM; MICOS; MINOS; MLCL; Membranes; MitOS; Mitochondria; OMM; PA; PAM; PC; PE; PG; PI; PS; Phospholipid; Protein complex; SAM; TIM; TOM; cardiolipin (diphosphatidylglycerol); contact sites; dimethylphosphatidylethanolamine; endoplasmic reticulum; inner mitochondrial membrane; intermembrane space; mitochondria associated membrane; mitochondrial contact site; mitochondrial inner membrane organizing system; mitochondrial organizing structure; monolysocardiolipin; outer mitochondrial membrane; phosphatidic acid; phosphatidylcholine; phosphatidylethanolamine; phosphatidylglycerol; phosphatidylinositol; phosphatidylserine; presequence translocase associated motor; sorting and assembly machinery; translocase of the inner membrane; translocase of the outer membrane.
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