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. Author manuscript; available in PMC: 2024 Sep 5.
Published in final edited form as: Cell Metab. 2024 Aug 22;36(9):1963–1978. doi: 10.1016/j.cmet.2024.07.024

Figure 2. Mitochondrial lipid biosynthesis and trafficking.

Figure 2.

Mitochondrial PC is generated at the ER via the Kennedy pathway and other mechanisms and imported into the OMM by StarD7 (it may also play a role in translocating PC from the OMM to the IMM). PE generated at the ER does not enter mitochondria. Rather, PS generated by PSS1 or PSS2 from PC or PE is imported into mitochondria and further into the IMM by Prelid3b. Mitochondrial PS is then converted to PE via PSD. As a precursor for CL, PA is imported from OMM into IMM by Prelid1. Mitochondrial PA undergoes a series of reactions, including TAMM41, PGS, PTPMT1, and CLS. Nascent CL produced by CLS is transacylated to form TLCL by TAFAZZIN and other de/reacylases. CMP, cytidine monophosphate; Gly, glycerol; Ser, serine; Etn, ethanolamine; Cho, choline.